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I had researched on prions and the Kuru disease for a class while looking at the impacts of animal testing (the Kuru disease is a prion found in New Guinea and is considered to be the first prion disease). Prions are scarily dangerous - near-fatal if exposed to it, and stable at much higher temperatures that regular non-misfolded proteins.

However, your chances of getting prions is very low. As long as you avoid "exotic" meat like monkey brains and stay in countries with strong regulatory bodies (like the US/EU etc) the risk of exposure is extremely limited. Bonus points if you avoid swimming in random bodies of water to not get contaminated water with brain-eating amoeba traveling up your nose.

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A few meat processing plants used compressed air to remove grey matter from animal skulls, and some did not provide adequate PPE to their workers. Several people started to show unique neurological symptoms that seemed to correlate with years of exposure to aerosolized pig brains. One person that I know of sued Hormel in 2008, and the lawsuit was dismissed with prejudice in 2009.

I've always wondered if the case had merit and we just did not have sufficient medical knowledge/techniques at the time to investigate the source, of if someone read about prions and self-diagnosed.

https://www.austindailyherald.com/2009/02/hormel-qpp-lawsuit...

'aerosolized pig brains'

Wow! Thanks for one of the most disgusting imaginations I had in a while! :-)

Haven’t seen a more relevant username to a comment on HN

> Several people started to show unique neurological symptoms that seemed to correlate with years of exposure to aerosolized pig brains

I thought those were thought to be autoimmune issues from the person's body learning to treat neurons as a threat? Or is that a separate thing? (Or outright discredited?)

Yeah they really are scary dangerous. The risk of exposure is low. There are some other important transmission channels to consider. As you noted on their temperature stability, prions aren't sterilized by normal medical sterilization procedures including autoclaving. So transmission of prions by surgical instruments can occur and is well documented. The conventional advice is that surgical instruments contaminated with prions need to be thrown out. It can also occur with organ transplants. Advice: don't read about this if you're scheduled for surgery. It's probably the smallest risk you'll encounter but it might really capture your imagination.

As noted in the article, prions are also responsible for 'mad cow disease', Bovine Spongiform Encephalopathy (BSE) as well as Creutzfeldt–Jakob disease. If you really want to get into prions you can start with the wikipedia entries for those. They're pretty incredible from a physics / biology standpoint.

The thing the article doesn't point out that is important to keep in mind is that CJD is a really awful neurological wasting disease and it's likely that in those seven years Émilie Jaumain and her family suffered terribly.

"prions are also responsible for 'mad cow disease', Bovine Spongiform Encephalopathy (BSE) as well as Creutzfeldt–Jakob disease" ...

and scrapie in sheep, and Chronic Wasting Disease in deer and other wild Cervidae in North America. The latter is increasingly common in the US, although its wide spread is recent enough that little is known as to whether it is being transmitted back to humans through hunting and processing of deer.

Yes, noticed signs about the deer, in stores all over rural Quebec..

And, not just deer, but various other even non-ungulate mammals. Maybe reptiles too, dunno if anybody is checking.

This sounds like a twist on the plot of World War Z but kicked off by a newly discovered, highly contagious rapid onset form of human prion disease spreading across the population and turning people into the living dead with Mad Human Disease (i.e. becoming homicidal and deranged while their brains rapidly deteriorate), the difference from zombies in this plot however is that even if you kill them to save your life, you risk exposing yourself to the mutated prions even after their death and nothing can limit your exposure, they are essentially radioactive to be around and their human form prions persist long after their body is gone, leeching into the ground, water, air when physically disturbed, etc.

Anyone care to modify my plot to make it spicier?

Something about the whole world almost completely focusing on another threat/pandemic while the zombie deers are silently lurking in the woods?

I like the premise, keep it going!

Re: the medical instrument - why isn't bleach used in such a case then, or even as a standard procedure before autoclaving? (Or does that just damage the metal instead?)

Bleach is like $1 and a lot of the instruments thrown away are hundreds of dollars, and the hospital administration knows both of these figures.

I'm assuming that bleach is insufficient for this purpose.

Idk about bleach, but if you look at some of the studies, 600deg treatment for over a day and similar protocols were being evaluated, and even those scales of energy weren't clearly enough.

Looks like bleach (sodium hypochlorite) plus autoclaving is a method recommended by the World Health Organization (WHO), for whatever that's worth. https://en.wikipedia.org/wiki/Prion#Sterilization

My great love is travelling (mostly outside the EU/US) and this often involves countries where swimming in random bodies of water is part of the standard itinerary.

I'm sure I'm not particularly unusual in this respect. Most of friends have similar preferences.

Good for you

Did you respond sarkily because you thought I was bragging or something? I'm not sure I understand why I provoked this reaction from you.

Doing that sort of travel is, for most citizens of the industrialized west, pretty unusual.

Jumping into random bodies of water in the industrialized west is not unusual at all however.

Yeah my girls loved swimming in my in-laws pond behind their house. This ruined that. Statistically almost zero chance it will happen, but the ghost of the idea is there.

Especially when you see micrographs of the little demon: https://www.newsweek.com/brain-eating-amoebae-strike-again-t...

I guess I should count myself lucky for the 'dangerous' things I got to do as a kid; it seems to be a thing of the past.

If you wanted to define 'survivorship bias' with a simple example, this would do it.

This really is a poor example. The vast majority of children who grew up doing "dangerous" activities are still alive and well. Survivorship bias is more often used when the chance of surviving is statistically lower than most people realize.

It's still survivorship bias. They're only happy about it because nothing bad occurred to them, while there really are kids that got sick and I bet they're much less positive about these dangerous things they got to do.

I didn't say it wasn't, just that it was a poor example of it as the vast majority of kids ended up okay.

Or Mercury handling, for another.

Maybe I overstated, they still swam, just a bit more freaked out.

if you know it's a ghost of an idea, then you should have the strength to overcome the irrational bias.

After all you almost certainly put them at more risk just driving them there.

Sure, its just like razorblades in the candy and roaming sex traffickers and all that stuff.

I didn't say they stopped swimming, but it just added some bullshit on top that I would have rather done without.

If they can afford to, they probably will.

Lots of Westerners travel to Asia/Africa/middle East/Latin America. (And yeah, sometimes they catch diseases).

Also, you have to think whether people who normally live in those countries (non westerners) get exposed and face the consequences regularly.

I'm genuinely confused by this. Most of my friends (pre-covid) would holiday fairly regularly outside of Eurpope. We're not especially wealthy or unusual as far as I know. I can't think of many people I know who only holiday in the EU.

South-East Asia is a hugely popular destination. Do we know different demographics?

The reference is to natural freshwater, especially if it is stagnant. Most travel clinics will give advice against swimming in ponds, etc.

The Amoeba is most problematic in warm fresh water bodies. However, a couple of years ago a woman got infected from a neti pot- scared the crap out of me[1]. I now always boil the water.

[1] https://www.cbsnews.com/news/woman-dies-from-brain-eating-am...

Just "having friends who holiday" is, alone, a separating factor, mate.

Only if you interpret me as in somehow trying to deny the existence of people poorer than me, which is not at all what I'm saying.

This has all gone a bit off track. To bring this back to the original context:

> Doing that sort of travel is, for most citizens of the industrialized west, pretty unusual.

"Pretty unusual" is what I'm disputing. I'd say it's far from "pretty unusual" for average, regular people to go on holiday outside Europe.

Going on holiday and swimming in the ocean is one thing. Swimming in remote freshwater ponds is wholly another.

I can think of at least two holidays where I swam in freshwater.

I do not doubt that. Meanwhile, I didn't. At issue is how common your experience is. We know it is not universal, p=1. We know it is not nil, p=0. So, is it close to 0.5? 0.1? 0.01? My guess is it is between 0.1 and 0.01.

"Strange women lyin' in ponds distributin' swords is no basis for a system of government!"

I'll grant the freshwater bit pushes the probability down a fair bit.

But the comment that I was responding to was "Doing that sort of travel is, for most citizens of the industrialized west, pretty unusual." which seems to be more about "outside of Europe" than it was "and swimming in freshwater. That was the part I found strange. I think there's a couple of countries with much lower than average travel (the US already mentioned and I seem to recall that the French tend to stay within their own borders more than the average)

It's safe to say we know different demographics. I think this is more of an EU thing than a US thing. Also, likely an upper middle class or greater thing. I'm from the US and even pre-covid, most Americans in the bottom 80% of incomes are not "holidaying" anywhere, domestic or overseas on a regular basis. A large percentage of the remainder stick to the US for the majority of their vacations. Pretty much everyone I know(mostly top 20% earners) who travels overseas more than once a decade or so is foreign born, or at least 2nd generation, and traveling to visit family.

Apparently 42% of Americans have passports now, but it was about half that just a decade ago, and I'd attribute most of the growth to immigration, not vacations. Anecdotal, I'd say in SEAsia the "foreigners" I encountered were like 80% European. In Central America, the cheapest/shortest flight from the US, it was still like 50% Europeans. Whenever we're finally able to visit my wife's family again, she's goin to have to get special permission to take unpaid vacation time or else change jobs in order to be get enough time to make the trip to Asia worthwhile. That's pretty typical here.

Most of those passports are for flying to/from vacation destinations in Mexico, as well.

If a lower class person in the US goes to Europe or Asia, or really anywhere except other than the US, Canada, or sometimes Mexico at most, a lot of us of us will probably think of them as the particularly adventurous sort.

We do have a fair amount of people who go to Mexico, but even then, It's extremely common to not go.... anywhere, unless you have money, and especially not go anywhere regularly, mostly because it's expensive and nobody can afford time off work even if they could afford plane tickets.

It's semi-possible, but as far as I can tell lot of people don't really save up and make it a priority.

I doubt it, because I do the same type of travel and many (or often most) of the other people at those locations are also from the “industrialized west”

Yes, this is called "selection bias". Only one person I've ever met has ever done anything like this, let alone regularly.

With "this" being "gone on holiday outside Europe"? Or are you from the States?

I wonder if this whole disagreement is actually about the fact that Americans tend to travel abroad less than those from other Western countries.

Then you are exposing yourself to a potential prion transmission vector, though it's probably an incredibly low risk.

Prion disease incidence is very low but most all cases in the US are the sporadic kind. It looks like there were over 500 deaths in the US from CJD in 2019. It’s not really about avoiding infected tissue (for CJD)

https://www.cdc.gov/prions/cjd/occurrence-transmission.html?...

Here’s some basic info and radiology https://radiopaedia.org/articles/creutzfeldt-jakob-disease?l...

I wonder if it's truly sporadic or if we just don't have the necessary patient history to know the source.

Also, our chance of getting prions is low, because if it wasn't, we'd be extinct.

True. But they don’t go away, they persist in nature. So theoretically, the risk continues to grow as they accumulate over time.

Theres also "Fatal insomnia" which is a prion disease that is hereditary.

https://www.merckmanuals.com/home/brain,-spinal-cord,-and-ne...

Wow... Only 40 people in the world have it. https://www.youtube.com/watch?v=4Zaz67IcLDY

So scary.

Nonsense, it is not like they are forever chemicals. They break down over time so it isn't like the global prion count keeps going up

I would guess they still deteriorate over years so the risk might actually remain stable.

> Bonus points if you avoid swimming in random bodies of water to not get contaminated water with brain-eating amoeba traveling up your nose.

Also, be very careful with the water that you use in a neti pot.

The hormonal medication HCG is also a potential source as it’s made from human urine. I took it for years.

Global warming is expanding the range of naegleria fowleri, the brain-eating amoeba, in the US.

What are the risks of them being weaponized as biological weapons?

does the body not have protections against prions like heat shock protein?

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