U.S. Woman Dies from Mad Cow-Like Brain Disease That Lay Dormant for 50 Years
41 comments
·May 30, 2025toomuchtodo
SoftTalker
The 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).
KingMob
I 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.
chneu
Not 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-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/...
westmeal
What the fuck they made cows eat other cows? Dear God.
lithos
If you drive through farm country you see "fat" advertisements for finishing cattle or increasing milk production. IE: https://energyfeeds.com/magnafat/
Normal feed/grazing isn't optimal or cost effective compared to other options, for the animal's full lifecycle.
chneu
It used to be common place to grind it up and put it in the feed for animals.
Ranchers do a ton of shit that is pretty disgusting and messed up. If you call them out for it they'll get pretty upset and call you a commie or unamerican.
A lot of ranchers have "dead piles" where they just let the bodies rot. Dairy ops also routinely do the same stuff, along with just dumping milk on the ground or in "manure pits" which often leak into the groundwater.
I grew up in this world. Small town, ranching/farming, conservative. The stuff ranchers and dairy ops do is mindblowingly fucked up. They call it tradition and if you call them out you will be attacked.
A while back the governor of Colorado was like "Yo let's designate one day where people don't eat beef. It'll be good for the environment." Colorado ranchers lost their fucking minds. They threw huge hissy fits and ran campaigns to get people to consume more meat on that day. So dumb. Find some videos of the ranchers basically crying and throwing fits, they're pretty funny https://www.koaa.com/news/covering-colorado/communities-gath...
Freedom2
> A lot of countries, because of prion diseases, basically stopped importing US beef.
In many ways this is their loss. Yes, there is a risk, but US beef is the best in the world, with no small thanks to the rich multicultural society we live in.
chneu
Lol no it's not. Each country has specific tastes then breeds for it. American beef is not the same as Brazilian beef which isn't the same as European beef which isnt the same as Australian beef.
It's just preference. There is no "better".
I grew up ranching and have visited operations in several countries. It's all just preference. A lot of people think US beef isn't very good.
hollerith
I will buy grass-fed beef from NZ and Australia, but not from the US because of how often it contains toxins (probably mostly heavy metals, hard to say because I never had any analyzed, but I am unusually sensitive to heavy metals) in my personal beef-buying experience.
Grain-fed US beef doesn't have the problem.
My guess is that there has just been much more industrial activity in the US than in Aus and NZ.
Gigachad
Australian beef is way better, and doesn’t come loaded with prions.
dendrite9
I 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.
xeonmc
> I realized then that we wouldn't know for decades how many humans caught it.
Considering that the 90s is about 20 years ago, the timeline seems to match up given the social climate today.
danpalmer
This 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
I 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
It recently expired in the US as well
dwroberts
> likely contracted through contaminated human growth hormone (HGH) treatments she received as a child, they determined.
Important context for those unlikely to click through
tehlike
prion 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?)
flowerthoughts
Yeah, 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...)
rendall
Alzheimer’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.
topher515
I 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.
burnt-resistor
Sporadic (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
That 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
You 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.
AStonesThrow
Why mine for Bitcoin, when you can fold some prions at home?
gsf_emergency
For people who prefer vaccines to nightmare fuels, the following might offer some hope:
(2014)
https://nyulangone.org/news/first-successful-vaccination-aga...
ggm
Considering 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
PF?? 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
[flagged]
khana
[dead]
Tjerkienator
so everyone with dimentia that dies soon after diagnosis could be victim of mad cow disease?
Paper:
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://doi.org/10.3201/eid3106.241519