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U.S. Woman Dies from Mad Cow-Like Brain Disease That Lay Dormant for 50 Years
- toomuchtodo 1y agoPaper: Cadaveric Human Growth Hormone–Associated Creutzfeldt-Jakob Disease with Long Latency Period, United States - https://wwwnc.cdc.gov/eid/article/31/6/24-1519_article https://wwwnc.cdc.gov/eid/article/31/6/24-1519_article | https://doi.org/10.3201/eid3106.241519 https://doi.org/10.3201/eid3106.241519
- SoftTalker 1y agoThe paper makes it clear that the analysis of the prions showed it was iatrogenic (meaning it was caused by a medical treatment) and not something that she acquired by some other pathway such as eating meat or brain from an infected animal).
- tim333 1y agoI guess maybe the immune system protects a bit against the disease but weakens as you get older?
- deleted 1y ago[deleted]
- tehlike 1y agoprion diseases is one of the nightmare fuels. Alzheimer is one such disease, with no known cure, and as in this case, can lay dormant for years (thank god, i guess?)
- rendall 1y agoAlzheimer’s isn’t a prion disease, but it does show prion-like behavior. Misfolded proteins like amyloid-beta and tau can spread within the brain similarly to prions, but unlike true prion diseases (e.g. CJD), Alzheimer’s isn’t infectious and isn’t caused by prions. It's a distinct neurodegenerative disorder with overlapping mechanisms, not classification.
- flowerthoughts 1y agoYeah, they are the only diseases I really fear. There are few situations where a single molecule is enough to fuck up your entire brain in a way it can't even detect, and certainly not fight against. Don't eat animal brains. (And while we're at it: be cautious about livers, since some contain toxic levels of vitamin A...)
- SubiculumCode 1y agoI guess that means it could be made domant again maybe.
- agarren 1y agoThat doesn’t seem very likely from my reading. It sounds like it’s not unheard of for prion-diseases like Creutzfeldt-Jakob to take a while to destroy a person. The poor lady fta apparently had a genetic component that contributed to the latency. Brutal. Inducing dormancy in this and similar cases would effectively present a treatment, if not a cure, to an hereto untreatable, uncurable disease. > … the woman first visited doctors with tremors and trouble balancing. As is often the case, once symptoms started, her condition rapidly worsened. She was hospitalized … and several days into her stay, she fell into a coma that she would never awaken from. In other news, apparently hgh used to be extracted from cadavers. Wtlf.
- klipt 1y agoThey still get other stuff from cadavers. Like for gum transplant, they offer cadaver gum vs autologous gum taken from the patient's upper palate... I'd advise avoiding the cadaver gum. I assume they think it's safe or they wouldn't use it, but there could be unknown risks...
- dralley 1y agoTo be honest, it's a bit comforting to know that it happened quickly. Better in some ways than a long, slow degradation of the mind from which there's no recovery.
- khana 1y ago[dead]
- s3graham 1y ago> In other news, apparently hgh used to be extracted from cadavers. Creatine too.
- dwroberts 1y ago> likely contracted through contaminated human growth hormone (HGH) treatments she received as a child, they determined. Important context for those unlikely to click through
- KingMob 1y agoI remember when Mad Cow hit in the 90s. It seemed like not much happened, but one of the unmentioned little details was that the time frame of the disease matched the lifespan of the animal. I realized then that we wouldn't know for decades how many humans caught it.
- dendrite9 1y agoI happened to pick up How the Cows Turned Mad by Maxine Schwartz a couple weeks ago. I'm not finished with it yet, but in the section about Kuru there was a comment about how cases happened at least 4 decades after the cannibalism ended. I picked it up just to flip through but it is a pretty engrossing history of the various forms of prions disease.
- chneu 1y agoNot much happened to people but mad cow(prion diseases in general) completely changed US beef/cattle production. For example, it used to be common practice to feed dead cattle to the remaining cattle. That changed. Some ranchers still do it, though. We also changed the breeding programs and import/export. A lot of countries, because of prion diseases, basically stopped importing US beef. https://www.nytimes.com/2004/01/05/us/mad-cow-forces-beef-industry-to-change-course.html https://www.nytimes.com/2004/01/05/us/mad-cow-forces-beef-in... The US is also dealing with a chronic wasting disease issue in deer and other wild animals right now. It's one of those "about to explode" problems. https://www.aphis.usda.gov/livestock-poultry-disease/cervid/chronic-wasting https://www.aphis.usda.gov/livestock-poultry-disease/cervid/... https://pmc.ncbi.nlm.nih.gov/articles/PMC10892334/ https://pmc.ncbi.nlm.nih.gov/articles/PMC10892334/
- khana 1y ago[dead]
- gsf_emergency 1y agoFor people who prefer vaccines to nightmare fuels, the following might offer some hope: (2014) https://nyulangone.org/news/first-successful-vaccination-against-mad-cow-wasting-disease-deer https://nyulangone.org/news/first-successful-vaccination-aga...
- ggm 1y agoConsidering how hard it has been to clense against prions, this is very encouraging. I find it interesting that we struggle to decontaminate materials because of their resistance, but we might be able to train the body to deal with them.
- gsf_emergency 1y agoPF?? Intuition says our immune system isn't equipped to detect them (note how odourless/tasteless) but you make me wonder if we might evolve bacteria/archae to sense them, as a first milestone..
- whatsupdog 1y ago[flagged]
- Tjerkienator 1y agoso everyone with dimentia that dies soon after diagnosis could be victim of mad cow disease?
- danpalmer 1y agoThis is one reason why there are limits around the world on who can donate blood. I received a blood transfusion as a child in the UK in the 90s, and specifically because of it being that country/period, I will never be able to donate blood here in Australia.
- BLKNSLVR 1y agoI thought the time limit in Australia expired recently (as in: within the last two to three years). As of July 25, 2022, the Australian blood donation rules that previously barred individuals who lived in the UK for more than six months between 1980 and 1996 from donating blood have been lifted. Maybe worth double checking at your local blood donor centre. There always seems to be a shortage of blood.
- TrueGeek 1y agoIt recently expired in the US as well
- danpalmer 1y ago> individuals who lived in the UK I received a blood transfusion in the UK during this time. I saw this announcement a while back and checked, and I am still covered by this and unlikely to ever be able to donate blood. Most people don't receive transfusions, and it's much higher risk, so pretty much no reason to get rid of that limitation.
- AStonesThrow 1y agoWhy mine for Bitcoin, when you can fold some prions at home? https://en.wikipedia.org/wiki/Folding@home https://en.wikipedia.org/wiki/Folding@home
- burnt-resistor 1y agoSporadic (spontaneous) CJD is the most common form. It doesn't require contaminated food or medical products. Proteins can misfold all on their own. The risk is about 2 micromorts / year.
- JauntTrooper 1y agoThat is such a long latency period. I wonder if it slowly progresses over time, or if it develops opportunistically once some other bodily system that keeps it in check breaks down with age.
- water-data-dude 1y agoYou have one misfolded protein that “teaches” other proteins to misfold in the same way[1]. It’s very slow initially because you’re talking about individual proteins, and it takes a while for each prion to bump into a normal protein of the correct type. The immune system can’t do anything about them though, so once a protein is converted there’s permanently another prion. The conversion rate is very slow, but it never ever ratchets back - the number only increases. It’s a geometric progression. Once symptoms show up there’s a TON of them, and it goes downhill fairly quickly. That’s why there’s such a long latency period. [1]It’s like Ice-9, if you’ve read Cat’s Cradle.
- topher515 1y agoI recently read “The Family That Couldn’t Sleep” which is the story of the history and discovery of prion diseases. It’s from the mid-00’s so it’s missing the latest research but it’s still an extremely interesting (and terrifying) read.