1 00:00:01,120 --> 00:00:04,320 Speaker 1: Welcome to you stuff you should know from How Stuff 2 00:00:04,320 --> 00:00:12,680 Speaker 1: Works dot com. Hey, and welcome to the podcast. I'm 3 00:00:12,760 --> 00:00:17,520 Speaker 1: Josh Clark with Charleston, Chuck, Bryan and Jerry and this 4 00:00:17,600 --> 00:00:21,040 Speaker 1: is not a day for podcast. I feel like I 5 00:00:21,120 --> 00:00:27,360 Speaker 1: sound like um one of the the public radio gals 6 00:00:27,800 --> 00:00:32,440 Speaker 1: from the early two thousands. Saturday. Oh yeah, yeah, yeah, 7 00:00:32,440 --> 00:00:37,120 Speaker 1: I remember the uh shwetty balls. It's a classic bed 8 00:00:37,680 --> 00:00:40,720 Speaker 1: good times right the Yeah, I was feigning like I 9 00:00:40,760 --> 00:00:42,559 Speaker 1: was yawning, as if I had been up all night 10 00:00:42,560 --> 00:00:45,640 Speaker 1: with insomnia. That's oh, I thought it was because of 11 00:00:45,680 --> 00:00:48,040 Speaker 1: the weather outside. Just a little play acting I got 12 00:00:48,040 --> 00:00:49,720 Speaker 1: you to get us going. You know, it's weird. I 13 00:00:49,760 --> 00:00:53,920 Speaker 1: got a little tired just studying this. Yeah, it's crazy. 14 00:00:53,960 --> 00:00:57,200 Speaker 1: How suggestive How Stuff Works articles are. Well, yeah, not 15 00:00:57,320 --> 00:01:01,280 Speaker 1: in that way, No, no, on that way. I mean, like, 16 00:01:01,960 --> 00:01:06,320 Speaker 1: you know, suggestive in that way. Jerry did not get 17 00:01:06,319 --> 00:01:08,080 Speaker 1: any sleep last night. We were talking about it before 18 00:01:08,080 --> 00:01:11,000 Speaker 1: we recorded. She got like a couple of hours, like 19 00:01:11,040 --> 00:01:13,720 Speaker 1: one of those deals where you wake up and then 20 00:01:14,360 --> 00:01:16,640 Speaker 1: you just stay awake for hours and hours. And I 21 00:01:16,680 --> 00:01:18,320 Speaker 1: told her, I said, you may have been sleeping during 22 00:01:18,319 --> 00:01:21,080 Speaker 1: some of that time, because you know, sometimes you'll be 23 00:01:21,200 --> 00:01:23,800 Speaker 1: distressed about not sleeping, and you'd be like, man, I'm 24 00:01:23,800 --> 00:01:25,480 Speaker 1: just awake, and then you wake up and you're like, 25 00:01:25,880 --> 00:01:28,839 Speaker 1: was I just dreaming about being stressed about being awake? Yeah? Well, 26 00:01:28,840 --> 00:01:32,759 Speaker 1: what's the answer, Jerry, she's tired. I'm going to answer 27 00:01:32,760 --> 00:01:36,000 Speaker 1: for I think you should. But um, yeah, I don't 28 00:01:36,040 --> 00:01:38,760 Speaker 1: typically get insomnia, but sometimes I can psych myself out 29 00:01:38,800 --> 00:01:42,840 Speaker 1: a little bit mentally with about of it. And that's 30 00:01:42,840 --> 00:01:44,880 Speaker 1: when I'm laying there going like, oh man, not this. 31 00:01:45,480 --> 00:01:48,880 Speaker 1: Oh yeah, once you start thinking about it, it is over. 32 00:01:49,000 --> 00:01:54,400 Speaker 1: So that's that is what one might call typical standard insomnia. Yeah, 33 00:01:54,400 --> 00:01:56,520 Speaker 1: that's not what this is about. No, no, Well we're 34 00:01:56,560 --> 00:02:02,200 Speaker 1: talking about is a very very rare genetic disorder. Well 35 00:02:02,240 --> 00:02:05,480 Speaker 1: it's not even genetic disorder. It's a neurodegenerative disorder, I 36 00:02:05,480 --> 00:02:10,560 Speaker 1: guess is what you call it. It's called fatal familial insomnia. Fatal. 37 00:02:10,960 --> 00:02:12,520 Speaker 1: That should tell you all you need to know. This 38 00:02:12,600 --> 00:02:16,280 Speaker 1: is insomnia that will kill you exactly like without know 39 00:02:16,360 --> 00:02:18,560 Speaker 1: if sands are but you will die. And like I said, 40 00:02:18,560 --> 00:02:22,840 Speaker 1: it's very very rare, Chuck. They think that possibly a 41 00:02:22,919 --> 00:02:28,520 Speaker 1: hundred people since they started analyzing this or you noticed it. 42 00:02:28,840 --> 00:02:32,120 Speaker 1: I think in the eighteenth century probably have died from 43 00:02:32,200 --> 00:02:35,799 Speaker 1: fatil familial insomnia. So it's a very rare disease. And 44 00:02:35,880 --> 00:02:40,240 Speaker 1: but it's also you'll notice, familial. It's very frequently passed 45 00:02:40,240 --> 00:02:45,519 Speaker 1: down along family lines, almost always, so they think tops 46 00:02:45,919 --> 00:02:50,440 Speaker 1: forty families are touched by it. Yeah, and I already misspoke, 47 00:02:50,560 --> 00:02:54,440 Speaker 1: which will correct later. But I said it's insomnia that 48 00:02:54,480 --> 00:02:56,280 Speaker 1: will kill you, and that's not really a case. The 49 00:02:56,320 --> 00:02:59,680 Speaker 1: insomnia is just a symptom of a larger problem in 50 00:02:59,720 --> 00:03:03,160 Speaker 1: your that will kill you. Yes, but the insomnia does 51 00:03:03,240 --> 00:03:07,880 Speaker 1: not help. Sure, it makes everything worse. So Um, the 52 00:03:08,040 --> 00:03:10,680 Speaker 1: history of this is a little murky, but they have 53 00:03:10,800 --> 00:03:14,360 Speaker 1: traced it back to one of two people. One is 54 00:03:14,360 --> 00:03:17,079 Speaker 1: a guy who they just referred to as patient zero, 55 00:03:17,720 --> 00:03:22,239 Speaker 1: who possibly died in seventeen sixty five in Venice, Venice, Italy, 56 00:03:22,280 --> 00:03:25,520 Speaker 1: that is uh. And the other one is a guy 57 00:03:25,680 --> 00:03:32,560 Speaker 1: who died in eighteen thirty six, probably of fatal familial insomnia. Um, 58 00:03:32,600 --> 00:03:35,800 Speaker 1: also in Venice, Italy. What's going on over there, Well, 59 00:03:35,840 --> 00:03:42,880 Speaker 1: there's probably maybe so tainted pasta sauce, who knows what 60 00:03:43,040 --> 00:03:46,280 Speaker 1: what happened, But the whole thing, there are different ways 61 00:03:46,280 --> 00:03:51,520 Speaker 1: that it could get started Um, but the these whoever 62 00:03:52,040 --> 00:03:55,240 Speaker 1: the patient zero was, it's been passed down along their family. 63 00:03:56,080 --> 00:04:00,200 Speaker 1: Those two families especially have did not farewell over the RS. 64 00:04:01,040 --> 00:04:05,480 Speaker 1: It's very sad. It well, the diseases extremely sad. Yeah, 65 00:04:05,520 --> 00:04:07,680 Speaker 1: And like I said, it's not you're not dying just 66 00:04:07,720 --> 00:04:10,520 Speaker 1: because you can't sleep night after night. Um, that is 67 00:04:10,560 --> 00:04:13,760 Speaker 1: just a symptom. But what we're talking about in a 68 00:04:13,840 --> 00:04:17,440 Speaker 1: larger sense is something called a preon disease. These are 69 00:04:17,480 --> 00:04:22,240 Speaker 1: super rare and uh characteristic of a few things that 70 00:04:22,279 --> 00:04:26,040 Speaker 1: they call it a sponge AFM disease, which means you're 71 00:04:26,040 --> 00:04:28,960 Speaker 1: gonna get tiny little holes in your brain. You get 72 00:04:28,960 --> 00:04:34,600 Speaker 1: a sponge sponge brain and uh there is uh neural loss. 73 00:04:34,760 --> 00:04:37,599 Speaker 1: And one of the weird things is a failure to 74 00:04:37,680 --> 00:04:41,719 Speaker 1: induce inflammatory response, Yeah, which I guess is that's the 75 00:04:41,800 --> 00:04:45,720 Speaker 1: body first saying, hey, something's wrong here. I'm gonna puff up. 76 00:04:46,760 --> 00:04:49,680 Speaker 1: Whether it's an ankle sprain or you know, like a 77 00:04:49,720 --> 00:04:52,000 Speaker 1: disease that will that will make something in flame. That's 78 00:04:52,000 --> 00:04:54,919 Speaker 1: a sign of your body trying to fight something else. 79 00:04:55,240 --> 00:04:58,880 Speaker 1: There's actually a lot of controversy about prion diseases because 80 00:04:58,920 --> 00:05:03,880 Speaker 1: it doesn't make an any sense. It's an infectious agent, right, 81 00:05:04,320 --> 00:05:08,719 Speaker 1: But it's really just a misfolded protein. And it's really 82 00:05:08,800 --> 00:05:12,400 Speaker 1: bizarre because with any other kind of infection, you have 83 00:05:12,960 --> 00:05:17,080 Speaker 1: a viral infection, a bacterial infection, and a preon infection. 84 00:05:17,800 --> 00:05:22,880 Speaker 1: Viral infections and bacterial infections have DNA or RNA. A 85 00:05:22,880 --> 00:05:26,600 Speaker 1: protein again, is just a preon. It's just a misfolded protein, 86 00:05:26,680 --> 00:05:29,440 Speaker 1: so it shouldn't be able to infect anything, but it does. 87 00:05:30,480 --> 00:05:34,040 Speaker 1: So it's this crazy medical mystery that they're still trying 88 00:05:34,080 --> 00:05:36,239 Speaker 1: to get to the bottom of. But as they do, 89 00:05:36,720 --> 00:05:42,720 Speaker 1: diseases like fatal familial insomnia or um uh crips Field 90 00:05:42,960 --> 00:05:46,920 Speaker 1: Jacob's disease, which is like human mag cow disease. It's 91 00:05:46,960 --> 00:05:51,360 Speaker 1: a spongefformed disease, spongebrain. It happens with animals and humans. 92 00:05:51,360 --> 00:05:55,240 Speaker 1: I don't I think that's pretty important. So in in 93 00:05:55,240 --> 00:05:57,360 Speaker 1: in many ways, it makes a lot of sense that 94 00:05:57,839 --> 00:06:02,840 Speaker 1: you would be able to pass down this problematic um 95 00:06:02,839 --> 00:06:09,120 Speaker 1: it's an autosomal dominant neurodegenerative disease, fatal familial insomnia. It 96 00:06:09,160 --> 00:06:11,400 Speaker 1: makes sense that it would pass down along family lines 97 00:06:11,440 --> 00:06:17,320 Speaker 1: because there's a gene that UM in under normal circumstances, 98 00:06:17,320 --> 00:06:20,680 Speaker 1: expresses a protein the pr PC protein, which is a 99 00:06:20,680 --> 00:06:23,800 Speaker 1: normal protein. We don't know what it does, but we 100 00:06:23,880 --> 00:06:26,599 Speaker 1: do know that it probably ei there has to do 101 00:06:26,640 --> 00:06:31,680 Speaker 1: with copper ion transport into the cell. It prevents cellular 102 00:06:31,760 --> 00:06:36,200 Speaker 1: death until the time is right, or it helps create 103 00:06:36,440 --> 00:06:39,479 Speaker 1: the sheets around your nerve endings so you're not in 104 00:06:39,560 --> 00:06:43,560 Speaker 1: constant pain. They think it's one of those, right, But 105 00:06:43,640 --> 00:06:45,880 Speaker 1: because it's the brain, there's still some mystery. And I 106 00:06:45,880 --> 00:06:48,120 Speaker 1: don't even think it was like the midneties when they 107 00:06:48,120 --> 00:06:51,039 Speaker 1: finally name this, right, Yeah, so it's pretty new on 108 00:06:51,080 --> 00:06:53,360 Speaker 1: the scene as far as because it's so rare, right, 109 00:06:53,760 --> 00:06:56,760 Speaker 1: but not even just fatal familial insomnia. It wasn't until 110 00:06:56,800 --> 00:07:00,480 Speaker 1: two thousand five that they the these res riachers at 111 00:07:00,520 --> 00:07:05,240 Speaker 1: the University of Texas in Austin. Basically, ir irrefutally proved 112 00:07:05,560 --> 00:07:10,040 Speaker 1: that preons misfolded proteins are an infectious agent. Even though 113 00:07:10,040 --> 00:07:14,200 Speaker 1: we have no idea how this is happening, It's it's true. 114 00:07:14,320 --> 00:07:17,080 Speaker 1: There's three ways of getting an infection in A misfolded 115 00:07:17,080 --> 00:07:20,680 Speaker 1: protein is one of them. Yeah, like you said, they 116 00:07:20,680 --> 00:07:24,400 Speaker 1: can occur in three ways. One is um acquired and 117 00:07:24,440 --> 00:07:28,040 Speaker 1: that means you have an infection. Well, in the case 118 00:07:28,080 --> 00:07:30,200 Speaker 1: of kuru, which I guess we should talk about k 119 00:07:30,360 --> 00:07:33,160 Speaker 1: you are you? That is when you get an infection 120 00:07:33,480 --> 00:07:36,560 Speaker 1: because you ate someone's brain. Yeah, I think we talked 121 00:07:36,600 --> 00:07:39,280 Speaker 1: about that in the cannibalism episode, doesn't you. It seemed 122 00:07:39,320 --> 00:07:42,680 Speaker 1: familiar in Papua New Guinea in the fifties, Like I 123 00:07:42,720 --> 00:07:47,280 Speaker 1: guess a local I think British governor or agent started 124 00:07:47,320 --> 00:07:49,360 Speaker 1: noting that there is this thing that the four A 125 00:07:49,600 --> 00:07:54,120 Speaker 1: people who practiced funereal cannibalism that included the eatings of 126 00:07:54,160 --> 00:07:58,120 Speaker 1: the deceased brain, um, would come down with the disease 127 00:07:58,120 --> 00:08:02,000 Speaker 1: that they called kuru, which meant like trembling in fear right, 128 00:08:02,800 --> 00:08:05,600 Speaker 1: And they started investigating it a little more. And that's 129 00:08:05,600 --> 00:08:07,480 Speaker 1: when we started to get the idea that there was 130 00:08:07,520 --> 00:08:10,240 Speaker 1: such a thing as prion disease, that you could catch 131 00:08:10,720 --> 00:08:16,280 Speaker 1: a preon uh well disease from eating brain. Yeah, and 132 00:08:16,320 --> 00:08:18,760 Speaker 1: it would render them you know, they couldn't eventually walk 133 00:08:18,920 --> 00:08:21,440 Speaker 1: or talk or eat. They would just lose all their 134 00:08:21,480 --> 00:08:25,840 Speaker 1: motor function basically and waste away and die because they 135 00:08:25,840 --> 00:08:30,080 Speaker 1: can't swallow or chew or anything exactly. So um, that's 136 00:08:30,120 --> 00:08:33,640 Speaker 1: when we first started really noticing in humans this whole 137 00:08:33,640 --> 00:08:38,600 Speaker 1: idea of uh, spongebrain problems, and that's the acquired version. 138 00:08:39,400 --> 00:08:44,520 Speaker 1: There's also one called sporadic which basically you just all 139 00:08:44,559 --> 00:08:48,800 Speaker 1: of a sudden develop it was fatal familial insomnia, and 140 00:08:48,840 --> 00:08:50,920 Speaker 1: they don't know where it comes from or how it's caused. 141 00:08:51,640 --> 00:08:53,719 Speaker 1: But doesn't it doesn't the fact that like one of 142 00:08:53,760 --> 00:08:57,760 Speaker 1: those two, that there are those two different ways, and 143 00:08:57,800 --> 00:09:00,199 Speaker 1: I know there's a third one, but don't they make 144 00:09:00,200 --> 00:09:02,240 Speaker 1: you very suspicious of what the heck was going on 145 00:09:02,320 --> 00:09:05,840 Speaker 1: in Venice in the late eighteenth century? Totally because they 146 00:09:06,280 --> 00:09:10,760 Speaker 1: were both of those original patient zeros were unrelated, right yeah, 147 00:09:10,760 --> 00:09:13,800 Speaker 1: because one was seventeen sixty five was six. But even 148 00:09:13,840 --> 00:09:15,920 Speaker 1: still they if they were in the same family, they'd 149 00:09:15,960 --> 00:09:17,600 Speaker 1: be like, well, these guys are in the same family, 150 00:09:17,640 --> 00:09:20,240 Speaker 1: so probably the one from earlier. From what I've read, 151 00:09:20,280 --> 00:09:21,960 Speaker 1: they're not in the same family. They just happened to 152 00:09:21,960 --> 00:09:23,640 Speaker 1: live in the same town. I think that is correct. 153 00:09:23,679 --> 00:09:25,960 Speaker 1: So I wonder if people were eating some weird stuff 154 00:09:26,000 --> 00:09:31,800 Speaker 1: there and one of them sporadically developed fatal familial insomnia. 155 00:09:32,040 --> 00:09:35,680 Speaker 1: That's nutty. So there's a third way to right, yeah, inherited, 156 00:09:36,280 --> 00:09:40,200 Speaker 1: which is for as far as fatal familial insnia it's 157 00:09:40,240 --> 00:09:47,120 Speaker 1: almost always inherited UH from from your family's genetics. Almost always, 158 00:09:47,640 --> 00:09:50,319 Speaker 1: but not all prion diseases are, which is by the way, 159 00:09:50,360 --> 00:09:54,880 Speaker 1: it's derived from the words protein and infection. Yeah, there's 160 00:09:54,880 --> 00:10:01,439 Speaker 1: a guy in two who um coined the term UH 161 00:10:01,559 --> 00:10:04,080 Speaker 1: and I ended up winning the Nobel Prize, and I 162 00:10:04,120 --> 00:10:10,559 Speaker 1: think because of his early studies. Name was Stanley Pruss Prisoner. 163 00:10:11,080 --> 00:10:15,240 Speaker 1: He won the Nobel in nine um And even after 164 00:10:15,320 --> 00:10:17,680 Speaker 1: he won the Nobel, people are like, this is what 165 00:10:17,760 --> 00:10:20,480 Speaker 1: you're talking about is impossible. There's no way a protein 166 00:10:20,520 --> 00:10:24,360 Speaker 1: can infect other proteins. But that's exactly what happens. So, 167 00:10:24,840 --> 00:10:27,720 Speaker 1: like I said, there's a there's a normal protein, the 168 00:10:27,800 --> 00:10:31,720 Speaker 1: pr PC protein, right, and then there's the sponge aformed 169 00:10:31,840 --> 00:10:35,560 Speaker 1: version of it, the prp sc and that's after it's 170 00:10:35,559 --> 00:10:40,800 Speaker 1: been folded. Yes, that's the folded basically mutated version. So 171 00:10:41,440 --> 00:10:45,160 Speaker 1: the whole thing comes down to what's called a polymorphism 172 00:10:45,200 --> 00:10:49,880 Speaker 1: on a code on which is a sequence of nucleotides 173 00:10:50,240 --> 00:10:55,120 Speaker 1: amino acids on your DNA right on a gene, and 174 00:10:55,400 --> 00:10:59,680 Speaker 1: the specific code on these say three nucleotides say you 175 00:10:59,800 --> 00:11:02,920 Speaker 1: got eyes, mean that we're going to code this protein 176 00:11:03,240 --> 00:11:07,480 Speaker 1: and under normal circumstances. On this gene, the pr PC 177 00:11:07,679 --> 00:11:14,960 Speaker 1: protein is coded, but if you have a different nucleotide combination, 178 00:11:15,600 --> 00:11:21,040 Speaker 1: you start coding the pr p SC protein and that's 179 00:11:21,040 --> 00:11:25,559 Speaker 1: the misfolded one. Normal enough, right, Okay, Yes, we can 180 00:11:25,559 --> 00:11:29,280 Speaker 1: code abnormally folding proteins. It's what cancers and all that stuff. 181 00:11:29,720 --> 00:11:36,400 Speaker 1: The thing is, once the patient starts folding these proteins abnormally, 182 00:11:36,880 --> 00:11:42,360 Speaker 1: those proteins go in and somehow infect the already properly 183 00:11:42,400 --> 00:11:46,400 Speaker 1: folded proteins that were expressed in the brain elsewhere before. Yeah, 184 00:11:46,400 --> 00:11:49,360 Speaker 1: they bind to them, and they don't know how or why. 185 00:11:49,559 --> 00:11:53,040 Speaker 1: Isn't that bizarre? Yeah, it's totally bizarre. And once that happens, 186 00:11:53,160 --> 00:11:56,720 Speaker 1: you're in big trouble. You are in big trouble. And uh, 187 00:11:56,880 --> 00:11:59,200 Speaker 1: we'll get into some of the symptoms and stages right 188 00:11:59,240 --> 00:12:05,240 Speaker 1: after this message break. All right, So we are back 189 00:12:05,280 --> 00:12:09,840 Speaker 1: with some symptoms and stages. And this is in general 190 00:12:09,880 --> 00:12:13,560 Speaker 1: for all prion diseases. They're gonna share some symptoms, like 191 00:12:13,600 --> 00:12:18,240 Speaker 1: a handful of them. Um fatigue, it is one cognitive decline, 192 00:12:18,360 --> 00:12:22,480 Speaker 1: like you're gonna lose some memory. You might develop dementia. Uh, 193 00:12:22,720 --> 00:12:27,839 Speaker 1: rigidity with like movement and walking hallucinations. Um, but they 194 00:12:27,880 --> 00:12:30,320 Speaker 1: don't all have the same symptoms. You might get some 195 00:12:30,360 --> 00:12:32,600 Speaker 1: of these and not get others. Right. The hallmark of 196 00:12:32,600 --> 00:12:37,920 Speaker 1: fatal familial insomnia is appropriately enough insomnia. Yeah, and that's 197 00:12:38,000 --> 00:12:40,560 Speaker 1: like when you start. One of the really sad things 198 00:12:40,600 --> 00:12:43,680 Speaker 1: is once you start to notice that you're already although 199 00:12:43,679 --> 00:12:47,280 Speaker 1: they can't cure it anyway, but um, sometimes it's nice 200 00:12:47,280 --> 00:12:48,880 Speaker 1: to know these things early on, but once you start 201 00:12:48,920 --> 00:12:52,360 Speaker 1: noticing insomnia, it's pretty far along. Yeah. And most people 202 00:12:52,440 --> 00:12:55,200 Speaker 1: don't know that they have it early on because the 203 00:12:55,320 --> 00:12:59,200 Speaker 1: mean age of onset is fifty years old, and it's 204 00:12:59,240 --> 00:13:01,600 Speaker 1: kind of all over place, Like they have seen cases 205 00:13:01,640 --> 00:13:04,320 Speaker 1: as early as nineteen. They've seen him come on as 206 00:13:04,400 --> 00:13:07,400 Speaker 1: late as seventy two. You're doing pretty good. But for 207 00:13:07,440 --> 00:13:11,160 Speaker 1: the most part, it strikes you around late late forties, 208 00:13:11,240 --> 00:13:15,280 Speaker 1: early fifties, and once it happens, you've got between one 209 00:13:15,360 --> 00:13:19,760 Speaker 1: to maybe three years of basically a living hell before 210 00:13:19,800 --> 00:13:23,000 Speaker 1: you die of this. Yeah it Um. There's generally four 211 00:13:23,080 --> 00:13:26,400 Speaker 1: stages of f f i U, the first of which 212 00:13:26,480 --> 00:13:29,360 Speaker 1: is gonna start with the insomnia, and over about four 213 00:13:29,360 --> 00:13:32,240 Speaker 1: months is gonna get worse and worse. But like I said, 214 00:13:32,280 --> 00:13:35,480 Speaker 1: some people only notice it later on, UM, after other 215 00:13:35,920 --> 00:13:40,240 Speaker 1: UH symptoms become known, you might start to have like 216 00:13:40,320 --> 00:13:45,000 Speaker 1: panic attacks, phobia's paranoia. When you do manage to sleep, 217 00:13:45,120 --> 00:13:48,120 Speaker 1: you supposedly have like super vivid dreams, which is interesting, 218 00:13:48,280 --> 00:13:51,240 Speaker 1: but for the most part, you're having bouts of insomnia 219 00:13:51,360 --> 00:13:55,720 Speaker 1: big time. Stage two it gets even worse. This stage 220 00:13:55,800 --> 00:13:59,560 Speaker 1: lasts about five months on average or typically, which is 221 00:13:59,600 --> 00:14:02,199 Speaker 1: to say it has in less than a hundred people 222 00:14:02,240 --> 00:14:06,440 Speaker 1: in the history of Earth. UM. But the the you 223 00:14:06,600 --> 00:14:10,880 Speaker 1: enter this stage called sympathetic hyperactivity, which is where you're 224 00:14:10,920 --> 00:14:14,199 Speaker 1: just keyed up all the time. UM. Do you remember 225 00:14:14,280 --> 00:14:17,960 Speaker 1: we've talked about insomnia and sleep deprivation and a couple 226 00:14:18,000 --> 00:14:21,840 Speaker 1: of podcasts before and how just totally unhealthy it is. 227 00:14:22,280 --> 00:14:24,440 Speaker 1: One of the reasons it is so unhealthy is because 228 00:14:24,480 --> 00:14:29,520 Speaker 1: your body enters a state of constant stress reaction. And 229 00:14:29,560 --> 00:14:33,840 Speaker 1: that's what UM sympathetic hyperactivity is. It's like, UM, your 230 00:14:33,880 --> 00:14:37,000 Speaker 1: breathing is elevated, your heartbeat is elevated, your core body 231 00:14:37,040 --> 00:14:41,320 Speaker 1: temperature is elevated. UM, you're just tuned up all the 232 00:14:41,400 --> 00:14:44,800 Speaker 1: time and you're not getting the sleep to knock yourself 233 00:14:44,800 --> 00:14:48,520 Speaker 1: out of that state and to regroup and regenerate and rest. Yeah, 234 00:14:48,560 --> 00:14:50,960 Speaker 1: and you're gonna at this stage you're gonna have um 235 00:14:51,080 --> 00:14:55,400 Speaker 1: some memory loss, short term memory loss, mood changes, a 236 00:14:55,400 --> 00:14:59,040 Speaker 1: lot of anxiety and depression, and you're gonna start to 237 00:14:59,080 --> 00:15:02,080 Speaker 1: have some motor issues as well, like the way you 238 00:15:02,080 --> 00:15:04,560 Speaker 1: move and the way you walk. So things are starting 239 00:15:04,560 --> 00:15:06,440 Speaker 1: to get pretty bad at this point, and you're probably 240 00:15:06,520 --> 00:15:09,280 Speaker 1: pretty freaked out, especially if this doesn't run in your 241 00:15:09,280 --> 00:15:12,880 Speaker 1: family and you either acquired it or it's a sporadic case. 242 00:15:13,040 --> 00:15:15,280 Speaker 1: Well yeah, and you're not going to a doctor and 243 00:15:15,280 --> 00:15:17,400 Speaker 1: they're going, hey, this sounds like ff I to me 244 00:15:18,080 --> 00:15:21,280 Speaker 1: because I've never heard of it in my life exactly. Um, 245 00:15:21,360 --> 00:15:22,880 Speaker 1: well that's not true. Doctors have heard of this, but 246 00:15:22,960 --> 00:15:25,000 Speaker 1: you know what I mean, like two of them. Half. Yeah, 247 00:15:25,040 --> 00:15:27,240 Speaker 1: it's not the first go to I think when you 248 00:15:27,280 --> 00:15:30,240 Speaker 1: say I've been having trouble sleeping and I'm agitated, they're 249 00:15:30,280 --> 00:15:33,920 Speaker 1: probably gonna ask, like, what kind of drugs you've been doing? Yeah, 250 00:15:34,000 --> 00:15:37,120 Speaker 1: you know, like just lay off the pot. Well that 251 00:15:37,120 --> 00:15:40,320 Speaker 1: should make you sleep, though, right, I don't know. I 252 00:15:40,360 --> 00:15:42,960 Speaker 1: guess it probably depends on what type of pot. I 253 00:15:43,000 --> 00:15:45,240 Speaker 1: would think it's more I like lay off the speed. 254 00:15:46,000 --> 00:15:48,720 Speaker 1: Oh yeah, you know, I probably should be gone there 255 00:15:49,720 --> 00:15:53,320 Speaker 1: all right. Uh so the third stage is pretty short, 256 00:15:53,360 --> 00:15:57,280 Speaker 1: it's about three months long, and that's when you're uh 257 00:15:57,640 --> 00:16:01,400 Speaker 1: really delving into the hardcore. And so what's what's that 258 00:16:01,560 --> 00:16:05,840 Speaker 1: in stage one? In stage two, appropriately enough, you're sleeping, 259 00:16:06,360 --> 00:16:09,960 Speaker 1: but you're only entering stage one. In stage two of sleep, 260 00:16:10,760 --> 00:16:14,960 Speaker 1: which is stage one is considered um where you're just 261 00:16:15,480 --> 00:16:20,240 Speaker 1: very relaxed. Stage two is where you're starting to sleep, 262 00:16:20,240 --> 00:16:23,280 Speaker 1: but you can be woken up very easily, and you're 263 00:16:23,280 --> 00:16:26,120 Speaker 1: not Yeah, and you're not going beyond that. You're not 264 00:16:26,160 --> 00:16:29,440 Speaker 1: going into stage three or stage five, which is R 265 00:16:29,480 --> 00:16:32,840 Speaker 1: A M. Sleep. They combine stage three and stage four apparently. 266 00:16:33,360 --> 00:16:36,760 Speaker 1: But you're not getting to sleep. So by the third 267 00:16:36,880 --> 00:16:39,680 Speaker 1: stage of fatal familiar insomnia, you're not even going to 268 00:16:39,720 --> 00:16:43,080 Speaker 1: one or two. You're just not sleeping at all. And 269 00:16:43,160 --> 00:16:47,760 Speaker 1: it's been like this for um nine ten months already, 270 00:16:47,800 --> 00:16:50,960 Speaker 1: so you're just basically losing it at this point. Yeah, 271 00:16:51,000 --> 00:16:53,800 Speaker 1: and that will deliver you to the end stage stage four, 272 00:16:54,440 --> 00:16:57,200 Speaker 1: and it's called end stage. You know where you're headed there, 273 00:16:57,520 --> 00:17:03,440 Speaker 1: um serious decline in dementia, UM in brain activity. Maybe 274 00:17:03,480 --> 00:17:06,040 Speaker 1: you've got about six months at that point, but you're 275 00:17:06,040 --> 00:17:09,240 Speaker 1: going to lose the ability to speak and move. It's 276 00:17:09,280 --> 00:17:12,879 Speaker 1: called a kinetic mutism and basically fall into a comma 277 00:17:12,920 --> 00:17:16,720 Speaker 1: and death. So with with a kinetic mutism, you actually 278 00:17:16,720 --> 00:17:20,160 Speaker 1: have the ability to move and speak, but you lack 279 00:17:20,560 --> 00:17:25,600 Speaker 1: the basic will to do so. Apparently, like patients who 280 00:17:25,600 --> 00:17:28,560 Speaker 1: have come out of this have reported that will not 281 00:17:28,600 --> 00:17:31,120 Speaker 1: necessarily f FI because it's always fatal in a hundred 282 00:17:31,160 --> 00:17:34,000 Speaker 1: percent of cases. Um, But people who have had a 283 00:17:34,080 --> 00:17:37,720 Speaker 1: kinetic mutism for other reasons have said, like, like I 284 00:17:37,840 --> 00:17:40,560 Speaker 1: knew I could, but in any time I got the 285 00:17:40,600 --> 00:17:44,800 Speaker 1: will up to move, right, there was something else just 286 00:17:44,920 --> 00:17:47,840 Speaker 1: counteracting that that was stronger, and I just couldn't move 287 00:17:47,880 --> 00:17:51,679 Speaker 1: and couldn't talk. Yeah, and then like you said, you 288 00:17:51,760 --> 00:17:55,480 Speaker 1: go from that into a coma right, and then death. Yes, 289 00:17:55,600 --> 00:17:59,600 Speaker 1: and all prion diseases are fatal at this point and uncurable, 290 00:17:59,600 --> 00:18:03,040 Speaker 1: correct I guess? So yeah, yeah, as far as they know. So, Chuck, 291 00:18:03,160 --> 00:18:06,520 Speaker 1: what's what's going on in the brain here? Uh? Well, 292 00:18:06,560 --> 00:18:11,400 Speaker 1: basically your central nervous system is starting to break down. Um. 293 00:18:11,560 --> 00:18:16,960 Speaker 1: The anterior ventral and medio dorsal thalamic neurons. So those 294 00:18:16,960 --> 00:18:20,400 Speaker 1: are neurons in your thalamus that basically manage your motor functions, 295 00:18:20,960 --> 00:18:24,720 Speaker 1: they start to die out, and instead of being replaced 296 00:18:24,760 --> 00:18:26,720 Speaker 1: like your body likes to do when cells die out 297 00:18:26,760 --> 00:18:30,120 Speaker 1: with healthy ones, they don't your glial cells, which when 298 00:18:30,119 --> 00:18:32,119 Speaker 1: don't we talk about glial cells. We've talked about them 299 00:18:32,119 --> 00:18:35,080 Speaker 1: a few times. Yeah, we've in some brain wine before. 300 00:18:34,920 --> 00:18:39,800 Speaker 1: They're basically the cleaning service for your central nervous system. Uh, 301 00:18:39,840 --> 00:18:42,399 Speaker 1: and they help out with communication there they start to 302 00:18:42,440 --> 00:18:45,920 Speaker 1: die and form scar tissue and the thalamus and once 303 00:18:45,960 --> 00:18:49,480 Speaker 1: that happens, it's called gliosis. You've just got scar tissue 304 00:18:49,520 --> 00:18:52,400 Speaker 1: instead of healthy cells exactly, pretty rapid decline from there. 305 00:18:52,520 --> 00:18:57,240 Speaker 1: It kills the communication between cells. Right. Yeah. So, um, 306 00:18:57,280 --> 00:19:01,520 Speaker 1: with fatal familial insomnia like what just described as the 307 00:19:01,560 --> 00:19:05,720 Speaker 1: result of any spongeform prion disease, right, which again, we're 308 00:19:05,800 --> 00:19:08,000 Speaker 1: just tiny little holes in your brain. Right. So it 309 00:19:08,080 --> 00:19:13,520 Speaker 1: seems like the distinction between the different spongeyform diseases is 310 00:19:13,760 --> 00:19:17,440 Speaker 1: what part of the brain specifically they attack. Fatal familial 311 00:19:17,480 --> 00:19:22,680 Speaker 1: insomnia they attack the thalamus, specifically the hypothalamus and specifically 312 00:19:23,000 --> 00:19:28,440 Speaker 1: the parts of the hypothalamus that help regulate sleep. Um, 313 00:19:28,480 --> 00:19:32,600 Speaker 1: And there's this part of your hypothalamus that is an 314 00:19:33,280 --> 00:19:35,760 Speaker 1: it has it creates what's what you could call an 315 00:19:35,800 --> 00:19:39,800 Speaker 1: anti waking system, to where not only are the neurons 316 00:19:39,800 --> 00:19:45,639 Speaker 1: shut off in one respect, in another, a bunch of 317 00:19:45,680 --> 00:19:49,280 Speaker 1: neurons that are off while you're asleep are on and 318 00:19:49,320 --> 00:19:53,840 Speaker 1: just keeping you asleep. So when you're waking normally, those 319 00:19:53,880 --> 00:19:57,800 Speaker 1: neurons are off, and when you're sleeping they're on. The 320 00:19:57,880 --> 00:20:01,720 Speaker 1: problem is, if you have fatal familiar insomnia, Uh, the 321 00:20:01,800 --> 00:20:06,160 Speaker 1: prions have eaten away at this system, and now all 322 00:20:06,160 --> 00:20:09,560 Speaker 1: of a sudden that that anti waking system that keeps 323 00:20:09,560 --> 00:20:11,920 Speaker 1: you asleep when you're asleep allows you to go to sleep, 324 00:20:11,960 --> 00:20:15,160 Speaker 1: to transition from one stage of sleep to deeper stage 325 00:20:15,160 --> 00:20:18,560 Speaker 1: of sleep is no longer active any longer. And so 326 00:20:18,640 --> 00:20:21,159 Speaker 1: the only thing that is active is your wakefulness and 327 00:20:21,200 --> 00:20:24,440 Speaker 1: it is on all the time. So you know you're 328 00:20:24,520 --> 00:20:27,840 Speaker 1: dying and there's this it's got to be some sort 329 00:20:27,840 --> 00:20:30,280 Speaker 1: of madness from not being able to sleep. It just 330 00:20:30,320 --> 00:20:34,679 Speaker 1: exacerbates everything, like that's just one of the symptoms. It is, 331 00:20:35,280 --> 00:20:37,880 Speaker 1: and it is a symptom, but it's also uh, it's 332 00:20:38,000 --> 00:20:43,000 Speaker 1: it's also directly related to the mechanism of this disease. Yeah, 333 00:20:43,000 --> 00:20:45,000 Speaker 1: and it's got to speed up the process because your 334 00:20:45,000 --> 00:20:47,280 Speaker 1: body is not getting the rest it needs exactly top 335 00:20:47,320 --> 00:20:50,520 Speaker 1: of everything else. And and that's the that's the devious 336 00:20:50,560 --> 00:20:52,680 Speaker 1: part of the whole thing is not only is your 337 00:20:52,680 --> 00:20:54,960 Speaker 1: body not getting the rest it needs, it's on all 338 00:20:55,000 --> 00:20:59,240 Speaker 1: the time. So it's it's kind of like a doubly 339 00:21:00,080 --> 00:21:04,359 Speaker 1: hardcore as far as diseases go. So, like we said, 340 00:21:04,440 --> 00:21:08,280 Speaker 1: diagnosing it is tough a because it's so rare. Uh 341 00:21:08,880 --> 00:21:13,160 Speaker 1: be the symptoms are, you know, they're always patient reported. 342 00:21:13,200 --> 00:21:16,040 Speaker 1: So like I said, a doctor is not gonna like 343 00:21:16,240 --> 00:21:18,760 Speaker 1: look at this first thing. Uh, You're gonna go in 344 00:21:18,840 --> 00:21:21,040 Speaker 1: with your family history and maybe get some blood test 345 00:21:21,119 --> 00:21:25,280 Speaker 1: on UM, an I exam, a spinal tap. You might 346 00:21:25,320 --> 00:21:27,320 Speaker 1: get an m R I or a pet scam or 347 00:21:27,600 --> 00:21:31,600 Speaker 1: pet scam pet scan you've never believed in, or an 348 00:21:31,600 --> 00:21:36,040 Speaker 1: e G which measures measures electrical activity in your brain. 349 00:21:36,560 --> 00:21:39,439 Speaker 1: But um, it's really tough to diagnose. Well, yeah, and 350 00:21:39,480 --> 00:21:42,000 Speaker 1: none of that is gonna work until you've already entered 351 00:21:42,040 --> 00:21:45,320 Speaker 1: that stage. Yeah, like you've already entered the first stage 352 00:21:45,359 --> 00:21:49,480 Speaker 1: at least, because those those tests show, oh, yeah, you 353 00:21:49,520 --> 00:21:53,359 Speaker 1: have insomnia. And then once they established yeah, you have insomnia, 354 00:21:53,440 --> 00:21:58,440 Speaker 1: then they have to further establish that it's fatal familial insomnia. Yeah. 355 00:21:58,680 --> 00:22:02,960 Speaker 1: And by this time, also, Chuck, it's say about fifty 356 00:22:03,040 --> 00:22:06,359 Speaker 1: year about fifty years old, you've probably already had kids. 357 00:22:06,840 --> 00:22:09,280 Speaker 1: And so now once you find out you have fatal 358 00:22:09,280 --> 00:22:12,119 Speaker 1: familiar in samnia, you're also terrified that you've passed it 359 00:22:12,160 --> 00:22:15,400 Speaker 1: onto your children, you get about. Yeah, and since it's 360 00:22:15,400 --> 00:22:20,359 Speaker 1: an autosomal dominant trait or disease condition, um, all you 361 00:22:20,400 --> 00:22:22,359 Speaker 1: need is one parent with it to pass it on 362 00:22:22,400 --> 00:22:25,719 Speaker 1: to you. Yeah. It's so sad. It's like, basically, what 363 00:22:25,760 --> 00:22:28,800 Speaker 1: am I dying of? Because you can't fix me? And 364 00:22:30,400 --> 00:22:32,439 Speaker 1: are my kids going to die this as well? And 365 00:22:32,480 --> 00:22:36,200 Speaker 1: then their kids? Very horrible disease. All Right, we're gonna 366 00:22:36,240 --> 00:22:39,960 Speaker 1: get into I guess, finish up with a few a 367 00:22:40,000 --> 00:22:42,280 Speaker 1: little information on a few more of these pre owned diseases, 368 00:22:42,600 --> 00:22:53,200 Speaker 1: right for this break all right, before we get onto 369 00:22:53,440 --> 00:22:56,280 Speaker 1: the other preonn diseases, we do need to talk about treatment. 370 00:22:56,800 --> 00:23:00,159 Speaker 1: Like we've said over and over, sadly, there is no 371 00:23:01,720 --> 00:23:05,320 Speaker 1: curative treatment, but there is palliative care, which basically means 372 00:23:05,320 --> 00:23:08,000 Speaker 1: we're gonna try and help you out as much as 373 00:23:08,040 --> 00:23:11,359 Speaker 1: we can to be comfortable as you die. Uh. And 374 00:23:11,960 --> 00:23:14,800 Speaker 1: weirdly one of the things that they are looking into 375 00:23:15,080 --> 00:23:19,680 Speaker 1: and trying is giving patients g h by the club 376 00:23:19,800 --> 00:23:22,720 Speaker 1: drug that you hear about to help people sleep, and 377 00:23:22,720 --> 00:23:25,199 Speaker 1: they're actually prescribing that in some certain cases. Yeah, and 378 00:23:25,240 --> 00:23:31,320 Speaker 1: they it apparently gives them quick, small, short bouts of sleep. 379 00:23:31,960 --> 00:23:37,080 Speaker 1: But that's I'm sure incredibly wonderful sleep. Nonetheless, yeah, it 380 00:23:37,160 --> 00:23:39,480 Speaker 1: might decrease your heart rate and body temperature a little 381 00:23:39,480 --> 00:23:43,000 Speaker 1: bit too, but um, at that point, you know, when 382 00:23:43,040 --> 00:23:45,320 Speaker 1: you're taking g HB to get twenty minutes of sleep, 383 00:23:45,920 --> 00:23:49,800 Speaker 1: you've got a pretty sad end coming very soon. It's 384 00:23:49,920 --> 00:23:53,480 Speaker 1: very sad. The other great hope is gene therapy, where 385 00:23:53,520 --> 00:23:59,639 Speaker 1: basically the delete the gene that's responsible for um making 386 00:23:59,680 --> 00:24:04,040 Speaker 1: this protein misfold and insert the correct version of it. 387 00:24:04,800 --> 00:24:07,000 Speaker 1: That's got to be the future of these cures, don't 388 00:24:07,040 --> 00:24:08,679 Speaker 1: you think. Yeah, that's gonna be the future of a 389 00:24:08,680 --> 00:24:12,200 Speaker 1: lot of cures when we can just rewrite the code 390 00:24:12,200 --> 00:24:16,320 Speaker 1: of our genes to you know, make it express properly. Yeah, 391 00:24:16,920 --> 00:24:19,960 Speaker 1: But until then, there's gonna be some problems for people 392 00:24:20,000 --> 00:24:25,520 Speaker 1: with spongeformed diseases because there's nothing you can do, including 393 00:24:25,520 --> 00:24:28,400 Speaker 1: with fatal familial insomni you can give somebody g HP. 394 00:24:28,640 --> 00:24:30,280 Speaker 1: That's about it. Should we go over a few more 395 00:24:30,280 --> 00:24:34,560 Speaker 1: of these? Yeah, they're all equally devastating. Um, I think 396 00:24:34,600 --> 00:24:37,720 Speaker 1: you did mention c J D quitz felt jacob or 397 00:24:37,920 --> 00:24:44,320 Speaker 1: yakup disease yaup. Maybe sure. Anytime I see j A 398 00:24:44,440 --> 00:24:46,600 Speaker 1: k O B, I think of the like you know, 399 00:24:46,760 --> 00:24:50,800 Speaker 1: German pronunciation. Yeah, it's probably right. This one's the most 400 00:24:50,800 --> 00:24:53,840 Speaker 1: prevalent um and it is a spontaneous occurrence, which is 401 00:24:53,880 --> 00:24:56,600 Speaker 1: really creepy. Well, you can get it too from eating it. 402 00:24:57,480 --> 00:25:01,040 Speaker 1: Oh is that? I think ten perc and as spontaneous 403 00:25:01,080 --> 00:25:04,320 Speaker 1: and the rest you get from eating it's acquired. Oh no, 404 00:25:04,440 --> 00:25:08,439 Speaker 1: it's just ten percent are inherited. Okay. So that's the one, 405 00:25:08,480 --> 00:25:12,280 Speaker 1: if I'm not mistaken, that was directly related to the 406 00:25:12,359 --> 00:25:14,959 Speaker 1: outbreak of mad cow. That's like human mad cow disease. 407 00:25:15,480 --> 00:25:18,760 Speaker 1: Remember back in the nineties with the mad cow outbreak. Well, 408 00:25:18,840 --> 00:25:21,600 Speaker 1: that all came from feeding a bunch of cattle a 409 00:25:21,600 --> 00:25:25,080 Speaker 1: lot of ground up beef that included cattle brains that 410 00:25:25,119 --> 00:25:28,199 Speaker 1: had pre on diseases in it, and so the cattle 411 00:25:28,280 --> 00:25:31,639 Speaker 1: got mad cow disease, and from eating that cattle we 412 00:25:31,720 --> 00:25:36,000 Speaker 1: got kuuz felled yakub Okay, so that's the human version. Yes, 413 00:25:36,119 --> 00:25:38,639 Speaker 1: and that's that kind of points out a huge problem 414 00:25:38,680 --> 00:25:43,840 Speaker 1: with preon since they're not biological, not not living in 415 00:25:43,880 --> 00:25:47,440 Speaker 1: the sense that we consider an effective agent typically like 416 00:25:47,600 --> 00:25:52,680 Speaker 1: a bacteria or a virus. There's no genetic information to destroy, 417 00:25:52,840 --> 00:25:56,159 Speaker 1: like using heat or bleach or whatever. It can't be 418 00:25:56,240 --> 00:25:58,639 Speaker 1: killed as I saw it put somewhere else. It's the 419 00:25:58,680 --> 00:26:02,000 Speaker 1: perfect pathogen. Yes, that's a mouthful. It is, but it's 420 00:26:02,040 --> 00:26:09,520 Speaker 1: also horribly scary. It is another spongeform diseases. Scrapy that 421 00:26:09,640 --> 00:26:12,760 Speaker 1: just sounds good. Yeah, that is just in goats and 422 00:26:12,840 --> 00:26:15,920 Speaker 1: cheap though, So humans don't need to worry about it. Um. 423 00:26:16,000 --> 00:26:18,520 Speaker 1: And it is also genetic and they have no evidence 424 00:26:18,600 --> 00:26:21,280 Speaker 1: right now that that humans can get it. No, we've 425 00:26:21,280 --> 00:26:24,280 Speaker 1: got enough to worry about. We talked about mad cow 426 00:26:24,400 --> 00:26:31,280 Speaker 1: that is officially called bob and spongebform uh encephalopathy. Nice, 427 00:26:31,400 --> 00:26:34,000 Speaker 1: Is that right? I think so? Yeah, UM, deer and 428 00:26:34,080 --> 00:26:37,879 Speaker 1: elk might get chronic wasting disease in the Western US. 429 00:26:38,280 --> 00:26:41,679 Speaker 1: If you see a deer that is um really skinny 430 00:26:41,680 --> 00:26:45,280 Speaker 1: and drooling and can't swallow, um, they may have chronic 431 00:26:45,320 --> 00:26:49,000 Speaker 1: wasting disease. I've seen that that humans can have that 432 00:26:49,119 --> 00:26:51,040 Speaker 1: as well, But um, I don't know if it's the 433 00:26:51,040 --> 00:26:53,399 Speaker 1: same version. No, it doesn't seem like it, because I 434 00:26:53,400 --> 00:26:56,720 Speaker 1: think it's in patients that have like uh, that have 435 00:26:56,840 --> 00:27:00,520 Speaker 1: died from AIDS. They'll often like waste away, like no 436 00:27:00,680 --> 00:27:03,960 Speaker 1: amount of nutrients will like keep them from just losing 437 00:27:04,040 --> 00:27:07,359 Speaker 1: weight until they basically just die from wasting away. But 438 00:27:07,440 --> 00:27:09,679 Speaker 1: I don't think it's related to a brain disease. And 439 00:27:09,720 --> 00:27:13,240 Speaker 1: then chuck also going back to um c j D, 440 00:27:13,720 --> 00:27:18,000 Speaker 1: which is what we call it now officially forever c 441 00:27:18,160 --> 00:27:20,959 Speaker 1: j D. Ok Um. There was an outbreak of it 442 00:27:21,119 --> 00:27:25,600 Speaker 1: in the eighties and no the nineties. These French doctors 443 00:27:25,640 --> 00:27:27,920 Speaker 1: were using it in a growth hormone that they were 444 00:27:27,960 --> 00:27:32,040 Speaker 1: giving as injections to kids who had stunted growth, and 445 00:27:32,080 --> 00:27:36,360 Speaker 1: they were getting them from pituitary glands harvested from humans 446 00:27:36,840 --> 00:27:40,800 Speaker 1: and sold along the black market. So basically a bunch 447 00:27:40,840 --> 00:27:45,960 Speaker 1: of dead people in Bulgaria had their pituitary glands removed 448 00:27:46,000 --> 00:27:49,520 Speaker 1: and sold the doctors in France who were using them. Yeah, 449 00:27:49,600 --> 00:27:53,119 Speaker 1: and these growth things or these growth hormone shots that 450 00:27:53,200 --> 00:27:55,520 Speaker 1: they were giving kids, and like a sixty or eighty 451 00:27:55,640 --> 00:28:02,960 Speaker 1: kids died the nineties. Yeah, yeah, it sounds like seventeen nineties. 452 00:28:03,200 --> 00:28:06,359 Speaker 1: So it's it's very weird because we go from not 453 00:28:06,480 --> 00:28:10,560 Speaker 1: even recognizing diseases like fatal familial insomnia as a disease 454 00:28:10,640 --> 00:28:14,560 Speaker 1: until the eighties, having an outbreak of mad cow disease, 455 00:28:14,720 --> 00:28:17,720 Speaker 1: having an outbreak of c j D, all within a 456 00:28:17,760 --> 00:28:21,520 Speaker 1: couple of decades, and all the while people are saying like, no, 457 00:28:22,400 --> 00:28:26,520 Speaker 1: preons can't exist. What you're saying is is it can't 458 00:28:26,560 --> 00:28:30,400 Speaker 1: be possible. And a lot of people tried to disprove 459 00:28:31,000 --> 00:28:35,320 Speaker 1: preons by saying, Okay, there are different types of scrapey, 460 00:28:36,000 --> 00:28:41,239 Speaker 1: and say one hasn't a different incubation period. In this 461 00:28:41,280 --> 00:28:43,719 Speaker 1: other one, you'll get this symptom, but you won't like 462 00:28:43,760 --> 00:28:48,000 Speaker 1: the The sheep will talk, will speak Russian, but in 463 00:28:48,040 --> 00:28:51,760 Speaker 1: this other form of scrapy they speak Swedish. And they're 464 00:28:51,800 --> 00:28:55,000 Speaker 1: saying like, this proves that these preons have some sort 465 00:28:55,040 --> 00:28:58,640 Speaker 1: of virus associated with them that we're just missing. Right, 466 00:28:59,560 --> 00:29:04,080 Speaker 1: Another group or another argument against preons was well, Alzheimer's 467 00:29:04,160 --> 00:29:09,680 Speaker 1: disease is technically a sponge ofform disease. It's a misfolded 468 00:29:09,680 --> 00:29:14,000 Speaker 1: proteins creating plaque build up scar tissue in the brain 469 00:29:14,520 --> 00:29:18,120 Speaker 1: that leads to all these same symptoms as say C J. D. Right, 470 00:29:19,120 --> 00:29:23,840 Speaker 1: it's not infectious. What's going on here? So they said 471 00:29:23,880 --> 00:29:25,680 Speaker 1: that there is one way to prove this, and that 472 00:29:25,840 --> 00:29:30,720 Speaker 1: is to create a pre on in a test tube 473 00:29:30,920 --> 00:29:35,600 Speaker 1: completely from from the whole cloth and put it in 474 00:29:35,640 --> 00:29:37,880 Speaker 1: someone's brain and put in a healthy brain. And that's 475 00:29:37,880 --> 00:29:39,560 Speaker 1: exactly what they did in two thousand and five at 476 00:29:39,560 --> 00:29:42,640 Speaker 1: the University of Texas, and it infected the other proteins. 477 00:29:42,760 --> 00:29:45,840 Speaker 1: That's right. So it's like, prions are this weird thing 478 00:29:45,880 --> 00:29:48,920 Speaker 1: that we didn't know that is now infectious, was even possible, 479 00:29:49,440 --> 00:29:53,320 Speaker 1: and we're finally wrapping our heads around it. Are spongy, 480 00:29:53,560 --> 00:29:56,600 Speaker 1: whole filled heads. I think this has gotta be not 481 00:29:56,760 --> 00:29:59,520 Speaker 1: this particular, but uh, this has gotta be what's gonna 482 00:29:59,560 --> 00:30:01,920 Speaker 1: wipe out the human race one day. I don't know. 483 00:30:02,080 --> 00:30:04,720 Speaker 1: Bowl is making a pretty good case for itself lately. No, 484 00:30:04,840 --> 00:30:07,640 Speaker 1: that's what I'm saying, Just some disease, Like, Uh, I 485 00:30:07,680 --> 00:30:10,720 Speaker 1: don't think we're gonna blow ourselves up with or like 486 00:30:10,840 --> 00:30:13,200 Speaker 1: run out of food or blow ourselves up with nuclear bombs. 487 00:30:13,240 --> 00:30:15,480 Speaker 1: I think it's just gonna be another like weird plague 488 00:30:15,520 --> 00:30:18,200 Speaker 1: or something that we don't understand due to our close 489 00:30:18,240 --> 00:30:22,560 Speaker 1: association with live Stock, and it's not medtime soon. I 490 00:30:22,560 --> 00:30:27,719 Speaker 1: don't know. Yeah, you're worried. I'm not worried. I'm just more, 491 00:30:27,760 --> 00:30:30,200 Speaker 1: you know, realistic. You give your safe room, you're building 492 00:30:30,200 --> 00:30:32,360 Speaker 1: in the side of a mountain. No, that would indicate 493 00:30:32,360 --> 00:30:36,200 Speaker 1: that I was worried. Okay, I'm building mine. You got anything, well? 494 00:30:36,240 --> 00:30:40,840 Speaker 1: Can I come over? Good? Are you got anything else? No? Okay, Well, 495 00:30:40,880 --> 00:30:45,040 Speaker 1: that's fatal familial insomnia and prions. If you want to 496 00:30:45,080 --> 00:30:47,120 Speaker 1: learn more about those things, you can type those words 497 00:30:47,200 --> 00:30:50,000 Speaker 1: into the search bar at how stuff works dot com. 498 00:30:50,040 --> 00:30:52,080 Speaker 1: And since I said that, it's time for a listener mail, 499 00:30:53,520 --> 00:30:56,640 Speaker 1: and they call this Canadian email from a Canadian kid 500 00:30:57,000 --> 00:30:59,800 Speaker 1: from Canadia. From Canadia, Hey guys, my name is Ben 501 00:30:59,800 --> 00:31:02,280 Speaker 1: matt as him a seventeen year old Canadian A. He 502 00:31:02,360 --> 00:31:05,520 Speaker 1: said that I didn't add that. Then that makes me 503 00:31:05,560 --> 00:31:08,960 Speaker 1: think he's probably not Canadian. I've been well, he was 504 00:31:09,000 --> 00:31:11,000 Speaker 1: in jest. I think I've been working my way through 505 00:31:11,000 --> 00:31:12,920 Speaker 1: the backlog of episodes, and I recently listened to the 506 00:31:12,960 --> 00:31:15,440 Speaker 1: episode on serial Killers. And then he spoke of a 507 00:31:15,480 --> 00:31:19,400 Speaker 1: man who killed by feeding his victims to pigs picked 508 00:31:19,480 --> 00:31:23,720 Speaker 1: in huh, rob is that his name? Do you remember 509 00:31:23,720 --> 00:31:26,080 Speaker 1: that because it sounds like pig pen. No, he's a 510 00:31:26,080 --> 00:31:30,160 Speaker 1: pretty famous serial killer from Vancouver. This reminded me of 511 00:31:30,200 --> 00:31:32,480 Speaker 1: a true story from my dad's childhood on a farm 512 00:31:32,480 --> 00:31:36,160 Speaker 1: in Holland, Michigan. While he was growing up, he was 513 00:31:36,200 --> 00:31:39,280 Speaker 1: often given the job of feeding pigs, a job he despised. 514 00:31:39,560 --> 00:31:41,680 Speaker 1: Much of the stem back to a horrible accident that 515 00:31:41,880 --> 00:31:44,720 Speaker 1: happened to a nearby farmer. When this particular farmer was 516 00:31:44,760 --> 00:31:46,320 Speaker 1: out in his barn feeding pigs, he had a heart 517 00:31:46,320 --> 00:31:49,760 Speaker 1: attack and collapsed among them. When his family found him 518 00:31:49,760 --> 00:31:51,480 Speaker 1: a few hours later, all that remained of his body 519 00:31:51,520 --> 00:31:53,760 Speaker 1: were the palms of his hands and the soles of 520 00:31:53,840 --> 00:31:58,640 Speaker 1: his feet. Hate those parts. This experience understandably and stilled 521 00:31:58,640 --> 00:32:00,280 Speaker 1: fearing to my dad for many years to come. And 522 00:32:00,320 --> 00:32:03,479 Speaker 1: to be honest, I don't blame him anyway. Thanks for you, guys, 523 00:32:04,200 --> 00:32:06,160 Speaker 1: Thanks for all you do. A shout out would make 524 00:32:06,160 --> 00:32:10,160 Speaker 1: my day, maybe even my year. So been masked, shout out, 525 00:32:10,360 --> 00:32:13,720 Speaker 1: shout out. Thanks for the email. Nice Thanks a lot. Ben. 526 00:32:14,800 --> 00:32:18,280 Speaker 1: You haven't heard much about Robert Pickton. I don't think so. 527 00:32:18,520 --> 00:32:22,400 Speaker 1: He was operating in like the nineties. I think and 528 00:32:22,440 --> 00:32:25,160 Speaker 1: he was just like having prostitutes over and then he'd 529 00:32:25,200 --> 00:32:29,560 Speaker 1: murder him and do horrible stuff like stuff like that. 530 00:32:29,960 --> 00:32:32,160 Speaker 1: And he was supplying the public because you know, he 531 00:32:32,200 --> 00:32:34,720 Speaker 1: had a pig farm. He's applying the public with pork. 532 00:32:35,360 --> 00:32:37,560 Speaker 1: And they think that he ground up people in it 533 00:32:37,680 --> 00:32:39,560 Speaker 1: to the pork ate that and then people ate the pork. 534 00:32:40,440 --> 00:32:44,600 Speaker 1: No no, no, like he ground up people and mixed 535 00:32:44,600 --> 00:32:46,640 Speaker 1: it together with ground pork and then sold that at 536 00:32:46,640 --> 00:32:49,680 Speaker 1: his ground pork. Oh wow, it was even worse. This 537 00:32:49,760 --> 00:32:51,920 Speaker 1: is the nineteen nineties. He was a bad man. Where 538 00:32:52,000 --> 00:32:56,680 Speaker 1: was he Vancouver? Jeez? I know, I thought those people 539 00:32:56,720 --> 00:33:02,080 Speaker 1: are nice. Not Robert picked him. Man horrific. So if 540 00:33:02,160 --> 00:33:06,080 Speaker 1: you like Ben masked shout out, I want to get 541 00:33:06,120 --> 00:33:08,240 Speaker 1: in touch with Chucker me. You can tweet to us 542 00:33:08,280 --> 00:33:10,920 Speaker 1: at s y s K podcast. You can join us 543 00:33:10,960 --> 00:33:13,080 Speaker 1: on Facebook dot com, slash stuff you Should Know. You 544 00:33:13,120 --> 00:33:15,800 Speaker 1: can send us an email to Stuff Podcast at how 545 00:33:15,880 --> 00:33:18,360 Speaker 1: stuff Works dot com, and as always, joined us at 546 00:33:18,520 --> 00:33:26,120 Speaker 1: home on the web Stuff you Should Know dot com. 547 00:33:26,160 --> 00:33:28,680 Speaker 1: For more on this and thousands of other topics, visit 548 00:33:28,720 --> 00:33:37,280 Speaker 1: how Stuff Works dot com.