Stuff You Should Know - Is there a disease that kills by preventing sleep?

Episode Date: October 28, 2014

The strange disease of fatal familial insomnia was first recorded in the 18th century. Its victims lose their ability to sleep, slip into coma and die. The more we understand about FFI, the more myst...erious it becomes. Learn more about your ad-choices at https://www.iheartpodcastnetwork.comSee omnystudio.com/listener for privacy information.

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Starting point is 00:00:00 I'm Munga Shatikler and it turns out astrology is way more widespread than any of us want to believe. You can find it in Major League Baseball, International Banks, K-Pop groups, even the White House. But just when I thought I had a handle on this subject, something completely unbelievable happened to me and my whole view on astrology changed. Whether you're a skeptic or a believer, give me a few minutes because I think your ideas are about to change too.
Starting point is 00:00:26 Just a Skyline drive on the iHeart Radio app, Apple Podcast, or wherever you get your podcasts. Hey guys, it's Chikis from Chikis and Chill Podcast and I want to tell you about a really exciting episode. We're going to be talking to Nancy Rodriguez from Netflix's Love is Blind Season 3. Looking back at your experience, were there any red flags that you think you missed? What I saw as a weakness of his, I wanted to embrace. The way I thought of it was whatever love I have from you is extra for me. I already love myself enough.
Starting point is 00:00:59 Do I need you to validate me as a partner? Yes. Is it required for me to feel good about myself? No. Listen to Chikis and Chill on the iHeart Radio app, Apple Podcast, or wherever you get your podcasts. Welcome to Stuff You Should Know from HowStuffWorks.com. Hey and welcome to the podcast.
Starting point is 00:01:23 I'm Josh Clark with Charles W. Chuck Bryant and Jerry and this is Not A Day For Podcast. I feel like I sound like one of the public radio gals from the early 2000s Saturday Night Live. Oh yeah, yeah, yeah. Remember? The sweaty balls. Yeah, it's a classic vid. Yeah.
Starting point is 00:01:49 Good times. Right, them. I feel like I was yawning as if I had been up all night with insomnia. That's... Oh, I thought it was because of the weather outside. Nah, just a little play acting. I got you. Get us going.
Starting point is 00:02:00 You know, it's weird. I got a little tired just studying this. Yeah. It's crazy how suggestive how Stuff Works articles are. Well, yeah, not in that way. No. Not in that way. I mean, like, you know, suggestive, right, but not in that way.
Starting point is 00:02:16 Jerry did not get any sleep last night. We were talking about it before he recorded, she got like a couple of hours like one of those deals where you wake up and then you just stay awake for hours and hours. And I told her, I said, you may have been sleeping during some of that time because, you know, sometimes you'll be just stressed about not sleeping and you'd be like, man, I'm just awake. And then you wake up and you're like, was I just dreaming about being stressed about being awake?
Starting point is 00:02:39 Yeah. Well, what's the answer, Jerry? She's tired. Huh. I'm going to answer for her. I think you should. Um, yeah, I don't typically get insomnia, but sometimes I can psych myself out a little bit mentally with a bout of it.
Starting point is 00:02:53 And that's when I'm laying there going like, oh man, not this. Oh yeah. Once you start thinking about it, it is over. So that's, that is what one might call typical standard insomnia. Yeah. That's not what this is about. No, no. Well, we're talking about is a very, very rare genetic disorder.
Starting point is 00:03:13 Well, it's not even genetic disorder. It's a neurodegenerative disorder, I guess is what you call it. Yeah. It's called fatal familial insomnia. Fatal. That should tell you all you need to know. This is insomnia that will kill you. Yeah, exactly.
Starting point is 00:03:26 Like without no ifs ands or buts, you will die. And like I said, it's very, very rare, Chuck. Yeah. They think that possibly a hundred people since they started analyzing this or crazy noticed that I think in the 18th century probably have died from fatal familial insomnia. So it's a very rare disease. Yeah. And, but it's also, you'll notice familial, it's very frequently passed down along family
Starting point is 00:03:52 lines. Yeah, almost always. So they think tops 40 families are touched by it. Yeah. And I already misspoke, which will correct later, but I said it's insomnia that will kill you. And that's not really the case. Insomnia is just a symptom of a larger problem in your body that will kill you.
Starting point is 00:04:12 Yes. But the insomnia does not help. I'm sure it makes everything worse. So the history of this is a little murky, but they have traced it back to one of two people. One is a guy who they just referred to as patient zero, who possibly died in 1765 in Venice. Venice, Italy, that is.
Starting point is 00:04:33 Right. Yes. And the other one is a guy who died in 1836, probably of fatal familial insomnia. Also in Venice, Italy. What's going on over there? Well, there's probably cheese, maybe some tainted pasta sauce. Who knows what happened, but the whole thing, there are different ways that it could get started.
Starting point is 00:04:59 But whoever the patient zero was, it's been passed down along their family lines. Those two families, especially, did not fare well over the years. It's very sad. Well, the disease is extremely sad. Yes. And like I said, you're not dying just because you can't sleep night after night. That is just a symptom. But what we're talking about in a larger sense is something called a prion disease.
Starting point is 00:05:28 These are super rare and characteristic of a few things that they call it a spongiform disease, which means you're going to get tiny little holes in your brain, like a sponge. Sponge brain. And there is neural loss. And one of the weird things is a failure to induce inflammatory response, which I guess is that's the body first saying, hey, something's wrong here. I'm going to puff up, whether it's an ankle sprain or like a disease that'll make something inflame.
Starting point is 00:06:03 That's a sign of your body trying to fight something else. There's actually a lot of controversy about prion diseases because it doesn't make any sense. It's an infectious agent, but it's really just a misfolded protein. And it's really bizarre because with any other kind of infection, you have a viral infection and a bacterial infection and a prion infection. Viral infections and bacterial infections have DNA or RNA. A prion is just a misfolded protein.
Starting point is 00:06:37 So it shouldn't be able to infect anything, but it does. So it's this crazy medical mystery that they're still trying to get to the bottom of. But as they do, diseases like fatal familial insomnia or Crick's Field Jacobs disease, which is like human mag cow disease, it's a spongiform disease, sponge brain. Yeah, it happens with animals and humans. I don't think that's pretty important. So in many ways, it makes a lot of sense that you would be able to pass down this problematic it's a autosomal dominant neurodegenerative disease, fatal familial insomnia.
Starting point is 00:07:20 It makes sense that it would pass down along family lines because there's a gene that in under normal circumstances expresses a protein, the PRPC protein, which is a normal protein. We don't know what it does, but we do know that it probably either has to do with copper ion transport into the cell. It prevents cellular death until the time is right, or it helps create the sheets around your nerve endings so you're not in constant pain. Yeah. They think it's one of those, right?
Starting point is 00:07:53 Yeah, but because it's the brain, there's still some mystery. And I don't even think it was like the mid 1980s when they finally named this, right? Yeah. So it's pretty new on the scene as far as because it's so rare. Right. But not even just fatal familial insomnia. It wasn't until 2005 that these researchers at the University of Texas in Austin basically irrefutably proved that prions, misfolded proteins, are an infectious agent.
Starting point is 00:08:20 Even though we have no idea how this is happening, it's true. There's three ways of getting an infection and a misfolded protein is one of them. Yeah. Like you said, they can occur in three ways. One is acquired and that means you have an infection, well, in the case of Kuru, which I guess we should talk about, K-U-R-U, that is when you get an infection because you ate someone's brain. Yeah.
Starting point is 00:08:47 I think we talked about that in the cannibalism episode, don't you? It seemed familiar. In Papua New Guinea in the 50s, like I guess a local, I think British governor or agent started noting that there is this thing that the foray people who practiced funerial cannibalism that included the eatings of the deceased brain would come down with the disease that they called Kuru, which meant like trembling in fear. Yeah. Right?
Starting point is 00:09:14 Yeah. And they started investigating it a little more and that's when we started to get the idea that there was such a thing as prion disease, that you could catch a prion, well, disease from eating brain. Yeah, and it would render them, you know, they couldn't eventually walk or talk or eat. They would just lose all their motor function basically and waste away and die because they can't swallow or chew or anything. Exactly.
Starting point is 00:09:39 So, that's when we first started really noticing in humans this whole idea of sponge brain problems. Yeah. And that's the acquired version. Right. There's one called sporadic, which basically you just all of a sudden develop fatal familial insomnia. Yeah.
Starting point is 00:10:00 And they don't know where it comes from or how it's caused. No. But doesn't the fact that like one of those two, that there are those two different ways, and I know there's a third one, but don't they make you very suspicious of what the heck was going on in Venice in the late 18th century? Totally. Yeah, they were, both of those original patient zeros were unrelated, right? Yeah, because one was 1765, one was 1836.
Starting point is 00:10:24 But even still, if they were in the same family, they'd be like, well, these guys were in the same family. So, probably the one from earlier, from what I've read, they're not in the same family. They just happen to live in the same town. I think that is correct. So, I wonder if people were eating some weird stuff there and one of them sporadically developed fatal familial insomnia. That's nutty.
Starting point is 00:10:44 So, there's a third way too, right? Yeah, inherited, which is, as far as fatal familial insomnia, it's almost always inherited from your family's genetics, almost always. But not all prion diseases are, which is, by the way, is derived from the words protein and infection. Yeah, there was a guy in 1982 who coined the term and ended up winning the Nobel Prize in, I think, 1997 because of his early study. His name was Stanley Prusner.
Starting point is 00:11:22 He won the Nobel in 97. And even after he won the Nobel, people were like, this is what you're talking about is impossible. There's no way a protein can infect other proteins. But that's exactly what happens. So, like I said, there's a normal protein, the PRPC protein, right? And then there's the spongiform version of it, the PRPSC. Right, and that's after it's been folded?
Starting point is 00:11:47 Yes. That's the folded, basically mutated version. So, the whole thing comes down to what's called a polymorphism on a codon, which is a sequence of nucleotides, amino acids, on your DNA, right, on a gene. And the specific codon, these, say, three nucleotides, say, you guys mean that we're going to code this protein. And under normal circumstances, on this gene, the PRPC protein is coded. But if you have a different nucleotide combination, you start coding the PRPSC protein, and that's
Starting point is 00:12:31 the misfolded one. Normal enough, right? Yes, we can code abnormally folding proteins, it's what cancer is and all that stuff. The thing is, once the patient starts folding these proteins abnormally, those proteins go in and somehow infect the already properly folded proteins that were expressed in the brain elsewhere before. Yeah, they bind to them and they don't know how or why. Isn't that bizarre?
Starting point is 00:13:01 Yeah, it's totally bizarre. And once that happens, you're in big trouble. You are in big trouble, and we'll get into some of the symptoms and stages right after this message break. I'm Mangesh Atikala, and to be honest, I don't believe in astrology, but from the moment I was born, it's been a part of my life. In India, it's like smoking. You might not smoke, but you're going to get secondhand astrology.
Starting point is 00:13:26 And lately, I've been wondering if the universe has been trying to tell me to stop running and pay attention, because maybe there is magic in the stars if you're willing to look for it. So I rounded up some friends and we dove in, and let me tell you, it got weird fast. Tantric curses, Major League Baseball teams, canceled marriages, K-pop. But just when I thought I had to handle on this sweet and curious show about astrology, my whole world came crashing down. It doesn't look good, there is risk to father.
Starting point is 00:14:01 And my whole view on astrology, it changed. Whether you're a skeptic or a believer, I think your ideas are going to change too. Listen to Skyline Drive and the iHeart Radio app, Apple Podcast, or wherever you get your podcasts. Hey, I'm Lance Bass, host of the new iHeart podcast, Frosted Tips with Lance Bass. The hardest thing can be knowing who to turn to when questions arise or times get tough or you're at the end of the road. Ah, okay, I see what you're doing.
Starting point is 00:14:30 Do you ever think to yourself, what advice would Lance Bass and my favorite boy bands give me in this situation? If you do, you've come to the right place because I'm here to help. This I promise you. Oh, God. Seriously, I swear. And you won't have to send an SOS because I'll be there for you. Oh, man.
Starting point is 00:14:47 And so will my husband, Michael. Um, hey, that's me. Yep, we know that, Michael. And a different hot, sexy teen crush boy bander each week to guide you through life, step by step. Oh, not another one. Uh-huh. Kids, relationships, life in general can get messy.
Starting point is 00:15:01 You may be thinking, this is the story of my life. Just stop now. If so, tell everybody, yeah, everybody about my new podcast and make sure to listen so we'll never, ever have to say bye, bye, bye. Listen to Frosted Tips with Lance Bass on the iHeart radio app, Apple podcast or wherever you listen to podcasts. All right. So we are back with some symptoms and stages and, uh, this is in general for all pre-on
Starting point is 00:15:29 diseases, they're going to share some symptoms like a handful of them. Yeah. Um, fatigue is one, uh, cognitive decline, like you're going to lose some memory. You might develop dementia, uh, rigidity with like movement and walking hallucinations. Um, but they don't all have the same symptoms. You might get some of these and not get others. Right. So the fatal familial insomnia is appropriate enough for insomnia.
Starting point is 00:15:55 Yeah. And that's like when you start, one of the really sad things is once you start to notice that you're already, although they can't cure it anyway, no, but, um, sometimes it's nice to know these things early on, but once you start noticing insomnia, it's pretty far along. Yeah. And most people don't know that they have it early on, uh, because the mean age of onset is 50 years old.
Starting point is 00:16:16 Yeah. It's kind of all over the place, like they have seen cases as early as 19, they've seen them come on as late as 72, which you're doing pretty good, but for the most part, it strikes you around late, late forties, early fifties. And once it happens, you've got between one to maybe three years of basically a living hell before you die of this. Yeah. Um, there's generally four stages of, uh, FFI, uh, the first of which is going to start
Starting point is 00:16:45 with the insomnia and over about four months is going to get worse and worse. But like I said, some people only notice it later on, um, after other, uh, symptoms become known. Right. Uh, you might start to have like panic attacks, uh, phobias, paranoia, uh, when you do manage to sleep, you supposedly have like super vivid dreams, which is interesting. But for the most part you're having bouts of insomnia big time. Yeah.
Starting point is 00:17:11 And if you do, it gets even worse, this, um, stage lasts about five months on average or typically, which is to say it has in less than a hundred people in the history of earth. Sure. Um, but the, the, you enter this stage called sympathetic hyperactivity, which is where you're just keyed up all the time. Um, do you remember, we've talked about insomnia and sleep deprivation in a couple of podcasts before. Yeah.
Starting point is 00:17:38 And how just totally unhealthy it is. One of the reasons it is so unhealthy is because your body enters a state of constant stress reaction. Yeah. And that's what, um, sympathetic hyperactivity is. It's like, um, your breathing is elevated, your heartbeat is elevated, your core body temperature is elevated. Um, you're just tuned up all the time.
Starting point is 00:18:00 Yeah. And you're not getting the sleep to knock yourself out of that state and to regroup and regenerate and rest. Yeah. And at this stage, you're going to have, um, some memory loss, a short-term memory loss, mood changes, a lot of anxiety and depression. And you're going to start to have some motor issues as well, like, uh, the way you move and the way you walk.
Starting point is 00:18:21 Right. So things are starting to get pretty bad at this point. Yeah. And you're probably pretty freaked out, especially if this doesn't run in your family and you either acquired it or it's a sporadic case. Well, yeah. And you're not going to a doctor and they're going, Hey, this sounds like FFI to me because I've never heard of it in my life.
Starting point is 00:18:38 Exactly. Um, well, that's not true. Doctors have heard of this, but you know what I mean? Like two of them have. Yeah. It's not the, the first go to, I think when you say, I've been having trouble sleeping and I'm agitated, they're probably going to ask like what kind of drugs you've been doing.
Starting point is 00:18:51 Yeah. You know, just lay off the pot and you'll be fine. Well, that should make you sleep though, right? I don't know. I guess it probably depends on what type of pot. I would think it's more like lay off the speed. Oh yeah. You know, I probably should have gone there.
Starting point is 00:19:06 Yeah. All right. Uh, so the third stage is pretty short. It's about three months long and that's when you're, uh, really delving into the hardcore insomnia. Yeah. What's said in stage one and stage two appropriately enough, you're sleeping, but you're only entering stage one and stage two of sleep, right?
Starting point is 00:19:29 Which is stage one is considered, um, or you're just very relaxed. Yeah. Stage two is where you're starting to sleep, but you can be woken up very easily. Like Jerry. And you're not, yeah. And you're not going beyond that. You're not going into stage three or stage five, which is REM sleep. They combine stage three and stage four apparently, but you're not getting to sleep.
Starting point is 00:19:53 So by the third stage of fatal familial insomnia, you're not even going to one or two. Right. You're just not sleeping at all. And it's been like this for, um, nine, 10 months already. So you're just basically losing it at this point. Yeah. And that will deliver you to the end stage, stage four, uh, and it's called in stage, you know, where you're headed there, um, serious decline in dementia, um, in brain activity.
Starting point is 00:20:21 Maybe you've got about six months at that point, but you're going to lose the ability to speak and move. Yeah. That's called a kinetic mutism. You've basically fallen to a coma and death. So with, with a kinetic mutism, you actually have the ability to move and speak, but you lack the basic will to do so. Apparently like patients who've come out of this have reported that well, not necessarily
Starting point is 00:20:47 FFI because it's always fatal in a hundred percent of cases. Yeah. Um, but people who have had a kinetic mutism for other reasons have said like, I, like I knew I could, but in, in any time I'd got the will up to move, right? There was something else just counteracting that that was stronger and I just couldn't move and couldn't talk. Geez. Yeah.
Starting point is 00:21:09 And then like you said, you go from that into a coma. Right. And then death. Yes. And all pre-on diseases are fatal at this point and uncurable. Correct. I guess so. Yeah.
Starting point is 00:21:20 Yeah. As far as I know. So Chuck, what's, what's going on in the brain here? Well, basically your central nervous system is starting to break down the anterior ventral and mediodorsal thalamic neurons. So those are neurons in your thalamus that basically manage your motor functions. They start to die out and instead of being replaced like your body likes to do when cells die out with healthy ones, they don't your glial cells, which wouldn't we talk about
Starting point is 00:21:49 glial cells? We've talked about them a few times. Yeah. You've talked about them very normally before. They're basically the cleaning service for your central nervous system and they help out with communication there. They start to die and form scar tissue and the thalamus and once that happens, it's called gliosis, you've just got scar tissue instead of healthy cells.
Starting point is 00:22:08 Right. Exactly. It's a pretty rapid decline from there. It kills the communication between cells, right? Yeah. So with fatal familial insomnia, like what you've just described is the result of any spongiform prion disease, right? Yeah, which again, we're just tiny little holes in your brain.
Starting point is 00:22:25 Right. So it seems like the distinction between the different spongiform diseases is what part of the brain specifically they attack. Right. Fatal familial insomnia, they attack the thalamus, specifically the hypothalamus and specifically the parts of the hypothalamus that help regulate sleep. And there's this part of your hypothalamus that creates what you could call an anti-waking system to where not only are the neurons shut off in one respect, in another, a bunch
Starting point is 00:23:03 of neurons that are off while you're asleep are on and just keeping you asleep. So when you're waking normally, those neurons are off and when you're sleeping, they're on. So the problem is, if you have fatal familial insomnia, the prions have eaten away at this system. And now all of a sudden, that anti-waking system that keeps you asleep when you're asleep allows you to go to sleep. The transition from one stage of sleep to a deeper stage of sleep is no longer active
Starting point is 00:23:35 any longer. And so the only thing that is active is your wakefulness and it is on all the time. So you know you're dying and it's got to be some sort of madness from not being able to sleep. It just exacerbates everything. Yeah. And it's just one of the symptoms. It is.
Starting point is 00:23:53 And it is a symptom, but it's also directly related to the mechanism of this disease. Yeah, and it's got to speed up the process because your body's not getting the rest it needs on top of everything else. And that's the devious part of the whole thing, is not only is your body not getting the rest it needs, it's on all the time. So it's kind of like a doubly hardcore as far as diseases go. So like we said, diagnosing it is tough, A, because it's so rare. B, the symptoms are, you know, they're always patient reported.
Starting point is 00:24:31 So like I said, a doctor's not going to like look at this first thing. You're going to go in with your family history and maybe get some blood tests done. An eye exam, a spinal tap. You might get an MRI or a PET scan or a PET scan, or an EEG, which measures electrical activity in your brain. But it's really tough to diagnose. Well yeah, and none of that is going to work until you've already entered that stage. Like you've already entered the first stage at least because those tests show, oh yeah,
Starting point is 00:25:07 you have insomnia. And then once they establish, yeah, you have insomnia, then they have to further establish that it's fatal familial insomnia. And by this time also, Chuck, it's say about 50, you're about 50 years old. You probably already had kids. And so now, once you find out you have fatal familial insomnia, you're also terrified that you've passed it on to your children. Yeah, you get about a 50-50 chance.
Starting point is 00:25:32 Yeah, and since it's an autosomal dominant trait or disease condition, all you need is one parent with it to pass it on to you. Yeah, it's so sad. It's like, basically, what am I dying of because you can't fix me and are my kids going to die of this as well? Right. And then their kids. Very horrible disease.
Starting point is 00:25:53 All right, we're going to get into, I guess, finish up with a few, a little information on a few more of these pre-on diseases right after this break. I'm Mangesh Atikular, and to be honest, I don't believe in astrology, but from the moment I was born, it's been a part of my life. In India, it's like smoking. You might not smoke, but you're going to get second-hand astrology. And lately, I've been wondering if the universe has been trying to tell me to stop running and pay attention, because maybe there is magic in the stars if you're willing to look
Starting point is 00:26:29 for it. So I rounded up some friends and we dove in and let me tell you, it got weird fast. Tantric curses, Major League Baseball teams, canceled marriages, K-pop. But just when I thought I had to handle on this sweet and curious show about astrology, my whole world came crashing down. Situation doesn't look good. There is risk to father. And my whole view on astrology, it changed.
Starting point is 00:26:59 Whether you're a skeptic or a believer, I think your ideas are going to change too. The Skyline Drive and the I Heart Radio App, Apple Podcast, or wherever you get your podcasts. Hey, I'm Lance Bass, host of the new I Heart Podcast, Frosted Tips with Lance Bass. The hardest thing can be knowing who to turn to when questions arise or times get tough, or you're at the end of the road. Ah, okay, I see what you're doing. Do you ever think to yourself, what advice would Lance Bass and my favorite boy bands give me in this situation?
Starting point is 00:27:28 If you do, you've come to the right place because I'm here to help. This I promise you. Oh, God. Seriously. I swear. And you won't have to send an SOS because I'll be there for you. Oh, man. And so my husband, Michael.
Starting point is 00:27:41 Um, hey, that's me. Yep. We know that, Michael. And a different hot, sexy teen crush boy bander each week to guide you through life step by step. Oh, not another one. Uh-huh. Kids, relationships, life in general can get messy.
Starting point is 00:27:54 You may be thinking, this is the story of my life. Just stop now. If so, tell everybody, ya everybody, about my new podcast and make sure to listen so we'll never, ever have to say bye, bye, bye. Listen to Frosted Tips with Lance Bass on the iHeart Radio app, Apple podcast or wherever you listen to podcasts. All right, before we get on to the other prion diseases, we do need to talk about treatment. Like we've said over and over, sadly, there is no curative treatment, but there is palliative
Starting point is 00:28:29 care, which basically means we're going to try and help you out as much as we can to be comfortable as you die. And weirdly, one of the things that they are looking into and trying is giving patients GHB, the club drug that you hear about, to help people sleep, and they're actually prescribing that in certain cases. Yeah, it apparently gives them quick, small, short bouts of sleep. But that's, I'm sure, incredibly wonderful sleep nonetheless. Yeah, it might decrease your heart rate and body temperature a little bit too.
Starting point is 00:29:06 But at that point, you know, when you're taking GHB to get 20 minutes of sleep, you've got a pretty sad end coming. Very soon. It's very sad. The other great hope is gene therapy, where basically they delete the gene that's responsible for making this protein misfold and insert the correct version of it. That's got to be the future of these cures, don't you think? Yeah, that's going to be the future of a lot of cures when we can just rewrite the code
Starting point is 00:29:37 of our genes to make it expressed properly. But until then, there's going to be some problems for people with spongiform diseases, because there's nothing you can do, including with fatal familial insomnia. And you can give somebody GHB. That's about it. Should we go over a few more of these? Yeah. They're all equally devastating.
Starting point is 00:30:00 I think you did mention CJD, Kreuzfeldt, Jacob, or Iacop disease. Nice. Is it Iacop? Maybe? Sure. Anytime I see J-A-K-O-B, I think of the German pronunciation. Yeah, it's probably right. This one's the most prevalent, and it is a spontaneous occurrence, which is really creepy.
Starting point is 00:30:20 Well, you can get it, too, from eating it. Oh, is that really? Well, I think 10% is spontaneous, and the rest you get from eating. It's acquired. Oh, no, it's just 10% are inherited. Oh, okay. So that's the one, if I'm not mistaken, that was directly related to the outbreak of like human mag cow disease.
Starting point is 00:30:40 Oh, okay. You remember back in the 90s with the mag cow outbreak? Oh, yeah. Well, that all came from feeding a bunch of cattle a lot of ground up beef that included cattle brains. Yeah. That had prion diseases in it, and so the cattle got mag cow disease, and from eating that cattle, we got quartz filled Iacop.
Starting point is 00:30:59 Okay, so that's the human version? Yes, and that kind of points out a huge problem with prions, since they're not biological, not living in the sense that we consider an infective agent typically like a bacteria or a virus. Right. So, genetic information to destroy, like using heat or bleach or whatever, it can't be killed. As I saw it put somewhere else, it's the perfect pathogen, yes.
Starting point is 00:31:25 That's a mouthful. It is, but it's also horribly scary. It is. Another spongiform disease is scraping. That just sounds good, didn't it? Yeah. That is just in goats and sheep though, so humans don't need to worry about it, and it is also genetic, and they have no evidence right now that humans can get it.
Starting point is 00:31:46 No, we've got enough to worry about. We talked about mad cow. That is officially called bovine spongiform encephalopathy. Nice. Is that right? I think so, yeah. Deer and elk might get chronic wasting disease, and the Western U.S., if you see a deer that is really skinny and drooling and can't swallow, they may have chronic wasting disease.
Starting point is 00:32:11 I've seen that humans can have that as well, but I don't know if it's the same version. No, it doesn't seem like it, because I think it's in patients that have died from AIDS. They'll often waste away, and no amount of nutrients will keep them from just losing weight until they basically just die from wasting away, but I don't think it's related to a brain disease. And then Chuck, also going back to CJD, which is what we call it now officially forever. It's about Jacob. CJD.
Starting point is 00:32:44 Oh, okay. There was an outbreak of it in the 80s and, no, the 90s. These French doctors were using it in a growth hormone that they were giving as injections to kids who had stunted growth, and they were getting them from pituitary glands harvested from humans and sold along the black market. So basically, a bunch of dead people in Bulgaria had their pituitary glands removed and sold to doctors in France who were using them. That's crazy.
Starting point is 00:33:15 Yeah, and these growth things, or these growth hormone shots that they were giving kids in like 60 or 80 kids died. What year was this? The 90s. Wow. Yeah, the 1990s. Yeah. It sounds like 1790s.
Starting point is 00:33:28 So it's very weird, because we go from not even recognizing diseases like fatal familial insomnia as a disease until the 80s, having an outbreak of mag cow disease, having an outbreak of CJD, all within a couple of decades, and all the while people are saying, no, prions can't exist. What you're saying is it can't be possible, and a lot of people tried to disprove prions by saying, okay, there are different types of scrapie, and say one has a different incubation period. In this other one, you'll get this symptom, but you won't, like the sheep will talk, will
Starting point is 00:34:11 speak Russian, but in this other form of scrapie, they speak Swedish. And they're saying like, this proves that these prions have some sort of virus associated with them that we're just missing, right? Another group or another argument against prions was, well, Alzheimer's disease is technically a sponge-a-form disease. It's a misfolded protein, creating plaque buildup, scar tissue in the brain that leads to all these same symptoms as, say, CJD, right? It's not infectious.
Starting point is 00:34:47 What's going on here? So they said that there is one way to prove this, and that is to create a prion in a test tube completely from whole cloth. And put it in someone's brain. And put it in a healthy brain. Yeah. That's exactly what they did in 2005 at the University of Texas, and it infected the other proteins.
Starting point is 00:35:08 That's right. So prions are this weird thing that we didn't know. That is now infectious. Was even possible. Yeah. And we're finally wrapping our heads around it. Our spongy, whole-filled heads. I think this has got to be, not this particular, but this has got to be what's going to wipe
Starting point is 00:35:25 out the human race one day. I don't know. Ebola is making a pretty good case for itself lately. No, that's what I'm saying. Just some disease. Yeah. Like, I don't think we're going to blow ourselves up with, or run out of food or blow ourselves up with nuclear bombs.
Starting point is 00:35:38 I think it's just going to be another weird plague or something that we don't understand. Yeah. Due to our close association with livestock. And it's not going to be time soon. I don't know. Yeah? You worried? I'm not worried.
Starting point is 00:35:52 I'm just more, you know, realistic. Do you have your safe room you're building in the side of a mountain? No, that would indicate that I was worried. Oh, okay. I'm building mine. You got anything? Well, can I come over? Sure.
Starting point is 00:36:04 Good. You got anything else? No. Okay. Well, that's fatal familial insomnia and prions. If you want to learn more about those things, you can type those words into the search bar at howstuffworks.com and since I said that, it's time for a listener mail. We call this Canadian email from a Canadian kid.
Starting point is 00:36:22 From Canadian. From Canadian. Hey guys, my name is Ben Mast. I'm a 17-year-old Canadian, eh? He said that. I didn't add that. That makes me think he's probably not Canadian. I've been, well, he was in jest, I think.
Starting point is 00:36:35 I've been working my way through the backlog of episodes and I recently listened to the episode on serial killers. In it, you spoke of a man who killed by feeding his victims to pigs. Picked him. Huh? Robert picked him. Was that his name? Yeah.
Starting point is 00:36:50 Do you remember that because it sounds like pig pen? No, he's a pretty famous serial killer from Vancouver. This reminded me of a true story from my dad's childhood on a farm in Holland, Michigan. While he was growing up, he was often given the job of feeding pigs, a job he despised, much of the stem back to a horrible accident that happened to a nearby farmer. When this particular farmer was out in his barn feeding pigs, he had a heart attack and collapsed among them. Oh, no.
Starting point is 00:37:14 When his family found him a few hours later, all that remained of his body were the palms of his hands and the soles of his feet. Yeah, pigs hate those parts. This experience, understandably, instilled fear into my dad for many years to come and to be honest, I don't blame him. Anyway, thanks for you guys. Thanks for all you do. A shout out would make my day, maybe even my year.
Starting point is 00:37:34 So Ben Mast, shout out. Shout out. Thanks for the email. Nice. Thanks a lot, Ben. You haven't heard much about Robert Pickton? I don't think so. He was operating in the 1990s, I think, and he was just having prostitutes over and then
Starting point is 00:37:50 he'd murder them and do horrible stuff, like skin them and stuff like that. Yeah. He was supplying the public. He had a pig farm. He was supplying the public with pork. Oh, no. And they think that he ground up people in it, too. He'd pork ate that and then people ate the pork.
Starting point is 00:38:05 Wow. No, no, no. Like he ground up people and mixed it together with ground pork and then sold that as ground pork. Oh, wow. It was even worse. And this was in the 1990s? He was a bad man.
Starting point is 00:38:17 Where was he? Vancouver. Jeez. I know. I thought those people were nice. Not Robert Pickton, man. It's horrific. So if you, like, Ben Mast, shout out, want to get in touch with Chuck or me, you can tweet
Starting point is 00:38:33 to us at S-Y-S-K Podcast. You can join us on facebook.com slash stuffyoushouldknow. You can send us an email to stuffpodcast at housestuffworks.com. And as always, join us at our home on the web, stuffyoushouldknow.com. For more on this and thousands of other topics, visit housestuffworks.com. I'm Munga Chauticular and it turns out astrology is way more widespread than any of us want to believe. You can find it in Major League Baseball, international banks, K-pop groups, even the
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Starting point is 00:39:41 cult classic show, Hey Dude, bring you back to the days of slip dresses and choker necklaces. We're going to use Hey Dude as our jumping off point, but we are going to unpack and dive back into the decade of the 90s. We lived it and now we're calling on all of our friends to come back and relive it. Listen to Hey Dude the 90s called on the iHeart Radio app, Apple Podcasts, or wherever you get your podcasts.

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