Doctors investigate mystery brain disease in Canada(bbc.com)

315 pointsby aluket5 tahun yang lalu303 comments

Doctors investigate mystery brain disease in Canada

https://www.bbc.com/news/world-us-canada-56910393

311 comments

sirsinsalot5 tahun yang lalu

Prion diseases terrify me. Especially having been in the UK eating beef at the time of the BSE outbreaks in the 1990s.

I wonder if there have been studies on the scale of potential latent vCJD/Prion disease in the population?

phobosanomaly5 tahun yang lalu

Apparently the only options for diagnostic testing are brain biopsy and pulling CSF samples, both of which are probably a non-starter for screening purposes across a population.

Clinical Laboratory Tests Used To Aid in Diagnosis of Human Prion Disease Allyson Connor, Han Wang, Brian S. Appleby, Daniel D. Rhoads Journal of Clinical Microbiology Sep 2019, 57 (10) e00769-19; DOI: 10.1128/JCM.00769-19

https://jcm.asm.org/content/57/10/e00769-19

StavrosK5 tahun yang lalu

Did prions survive cooking? I'd have thought cooking the beef would have killed them.

Hypergraphe5 tahun yang lalu

Yes they do. From wikipedia : Prion aggregates are stable, and this structural stability means that prions are resistant to denaturation by chemical and physical agents: they cannot be destroyed by ordinary disinfection or cooking. This makes disposal and containment of these particles difficult.

rndude5 tahun yang lalu

Prions are incredible hard to destroy.

To quote from Wikipedia:

`Prion aggregates are stable, and this structural stability means that prions are resistant to denaturation by chemical and physical agents: they cannot be destroyed by ordinary disinfection or cooking.`

StavrosK5 tahun yang lalu

Oooof...

Red_Leaves_Flyy5 tahun yang lalu

Iirc hospitals that treat patients with prion diseases incinerate all materials that contact the patients. I don't know how they sanitize the rooms but it can't be cheap or eco friendly.

XorNot5 tahun yang lalu

Chemical agents which cause protein-crosslinking would be the preferred method - you can broadly make every side-group reactive and cause them all to stitch together with anything else organic, which tends to inactivate them (makes them a big blob which can't do much).

But it's nasty stuff to work with, since it also happily does tons of damage to the proteins making up the humans applying it.

Red_Leaves_Flyy5 tahun yang lalu

Do you have an example of such a chemical being used to effectively remediate prion contaminated environments?

XorNot5 tahun yang lalu

Apparently not: I was thinking glutaraldehyde which is used for cleaning in hospitals but reading up the advice for prions is explicitly opposed to cross-linking agents.

Which makes sense I guess but it's also scary.

Red_Leaves_Flyy5 tahun yang lalu

You raised my hopes and dashed them exquisitely.

Until we have better, cheaper, and less error prone methods of remediating prion diseases they're the #1 fear on my biological apocalypse list.

turing_complete5 tahun yang lalu

Destroying prions is eco friendly.

Red_Leaves_Flyy5 tahun yang lalu

Humans need a non toxic environment. Plants, and life in general, will find a way. With, and without, us.

iagovar5 tahun yang lalu

That's not good.

homero5 tahun yang lalu

Are prions contagious through contact or only eating?

Anon4Now5 tahun yang lalu

Sterrad sterilization machines, which use a hydrogen peroxide gas plasma, have been shown to be very effective against CJD (mad cow) and other prion diseases. However they are very expensive, and almost 20 years after their launch there are only 20k systems installed world wide.

ashildr5 tahun yang lalu

A plasma of H2O2 sounds like the definition of a reactive environment…

giantg25 tahun yang lalu

That's not entirely true. They know that bleach can be used against CWD for surface decontamination. I would call a 50/50 bleach water solution quite common for disinfectant.

https://www.nih.gov/news-events/news-releases/household-blea...

neartheplain5 tahun yang lalu

Yes, prions can survive cooking. Emphasis mine:

> 134 °C (273 °F) for 18 minutes in a pressurized steam autoclave has been found to be somewhat effective in deactivating the agent of disease

https://en.wikipedia.org/wiki/Prion#Sterilization

basicplus25 tahun yang lalu

Yes and they can pass on to other patients from surgical tools

https://www.scientificamerican.com/article/surgical-exposure...

petre5 tahun yang lalu

Yes they do, unfortunately. And there are recently discovered cases of chronic wasting disease (CWD) in elk, deer and moose worldwide. I hope it doesn't pass to humans. For the time being I'll refrain from eating game.

giantg25 tahun yang lalu

I would be more worried about beef. There has been no evidence of CWD jumping to humans, but there has been for BSE.

Also, do you have a link for the distribution? My understanding is that it is spread over a large geographic area, but that it's found in smaller clumps.

petre5 tahun yang lalu

There are several maps and studies, depending on which country you're interested in. The best one that I've found was for Norway. Needless to say, it's spreading over an increasingly larger area every year.

Yes, BSE is also worrying, but beef that you buy at supermarkets is also checked more thoroughly compared to game meat. I'd worry more about restaurants and their suppliers.

colechristensen5 tahun yang lalu

If you took affected neural tissue and burned it to ash on a wood fire, there remains (although low) infective potential but probably only if you were trying in a lab. It takes 1000 C incineration to defeat infectivity and even then they’re still detectable.

Composting so the proteins are broken down biologically is also an alternative.

No amount of cooking prions that leave anything you want to eat makes infected material safe.

giantg25 tahun yang lalu

Agree that cooking is not an option.

You can actually get that 1000C down to 600C if you also soak the tissue in acid prior to burning.

FredPret5 tahun yang lalu

That pretty much describes how I cook my steaks, a nice H2SO4 marinade followed by ten minutes in a bonfire

shoemakersteve5 tahun yang lalu

As long as you don't put ketchup on it.

robbiep5 tahun yang lalu

I’m not so sure about the composting - the breakdown of organic material (well, protein in this case) in soils is largely brought about by proteases and proteases are largely ineffective

jonnycomputer5 tahun yang lalu

I thought 200C for an hour was enough.

LorenPechtel5 tahun yang lalu

The only way to destroy prions is to destroy all protein in the material. What's left wouldn't be worth eating.

Hypergraphe5 tahun yang lalu

Yep, been there in the 90s, scary shit.

stormdennis5 tahun yang lalu

Many countries would not allow people who lived in the UK during the 80s and 90s to give blood. I ate so many burgers back then too. Our kids after they were born weren't allowed beef for years.

stuff4ben5 tahun yang lalu

Yep I'm one of them. Lived in the UK in the early 80's as a child. Gave blood for the first time when I was in college in the 90's to the Red Cross here in the US, and they mailed me back saying to never give blood again but didn't specify a reason. It wasn't until a few years later that I found out why. And the fact I might have a prion disease lurking in my blood sometimes terrifies me.

stormdennis5 tahun yang lalu

I don't think it's a concern any more. The trajectory of cases that should have occurred if CJD was widespread in the community, never happened. One consequence of this is the blood donation ban has been lifted in most countries, I think.

ed25519FUUU5 tahun yang lalu

Is there a blood test for that?

stuff4ben5 tahun yang lalu

I don't think so. I believe it was a screening question. Which was odd since they allowed me to give blood at that time.

wombatpm5 tahun yang lalu

Well they got your sample for testing going forward.

gadders5 tahun yang lalu

I lived through it. I mean, it was scary, but the risks were low.

I remember my landlord and his girlfriend at the time saying they were going to give up beef due to the risks. They said this whilst smoking cigarettes.

slashdev5 tahun yang lalu

People are so good at ignoring large daily risks and freaking out about tiny unknown risks.

Just look how many people are scared of the covid19 vaccines, but take bigger risks in their morning commute. Actually if you just stick to the AstraZenica vaccine with the risk of blood clots - you take a bigger risk just getting out of bed in the morning, even if you never leave the house.

dnautics5 tahun yang lalu

Yes and no. The vaccines are by definition new and untested. That the blood clots appeared suggests that the unknown surface area of the vaccine is higher than we expected; whereas the almost all of the risk on your commute are known and preventable.

For example let's say the vaccine causes a side effect that appears only two years from now; then you wouldn't necessarily be correct in asserting that the morning commute is more dangerous. By contrast, the morning commute causing an unknown side effect in two years is highly unlikely.

(For the record I'm vaccinated)

slashdev5 tahun yang lalu

The risks on your morning commute are not preventable by effort on your part. You can drive with care, but there is still substantial risk no matter what.

Unknown but remote risks with any vaccine are really quite substantially less dangerous than the known and quantifiable risk of your commute. We're talking like three orders of magnitude or more which leaves a lot of room for unknown risks.

dnautics5 tahun yang lalu

> Unknown but remote risks with any vaccine are really quite substantially less dangerous

How the hell can you say anything with confidence about an "unknown risk"? It's unknown.

tauntz5 tahun yang lalu

Prions terrify me as well because it seems like there's no stopping them..

> In 2015, researchers at The University of Texas Health Science Center at Houston found that plants can be a vector for prions. When researchers fed hamsters grass that grew on ground where a deer that died with chronic wasting disease (CWD) was buried, the hamsters became ill with CWD, suggesting that prions can bind to plants, which then take them up into the leaf and stem structure, where they can be eaten by herbivores, thus completing the cycle. It is thus possible that there is a progressively accumulating number of prions in the environment

Xcelerate5 tahun yang lalu

I had read this before. It's horrifying. How do you stop the chain reaction of proteins folding into lower energy states? It's not like a bacteria or virus; it's more like ice-nine, except instead of water it's your brain.

AbrahamParangi5 tahun yang lalu

Prions aren’t new, they’re probably as old as proteins- and they haven’t ice-nined the world yet.

HDMI_Cable5 tahun yang lalu

To be fair, neither did ice-nine until somebody decided to eat it.

anotha15 tahun yang lalu

To save someone else the time, the "ice-nine" referred to in this comment is fictional.

fao_5 tahun yang lalu

To be fair, "Cat's Cradle" is a famous work of science-fiction

Izkata5 tahun yang lalu

However, "ice IX" is real and distinct from "Ice-nine", so clarity is worthwhile.

https://en.m.wikipedia.org/wiki/Ice_IX

Also I only know of Ice-nine because of fun facts like this, didn't even know the name of the story let alone have read it.

intrasight5 tahun yang lalu

Seminal work of sci-fi, I highly recommend if for no other reason than to get the ice-nine references you'll inevitably come across.

Xcelerate5 tahun yang lalu

The funny thing is when I posted that, I hadn’t heard of Cat’s Cradle; I was just familiar with what the fictional ice-nine referred to.

anotha15 tahun yang lalu

For which generation?

fao_5 tahun yang lalu

Are there many noteworthy modern science fiction authors that hold up to the Asimov / Clarke / Lem generation? I would think someone reasonably versed in science fiction would have at least heard of it -- I'm a "zoomer" and I have.

intergalplan5 tahun yang lalu

The actual writing from a lot of the golden age and New Wave sci-fi authors is... OK at best, with a few exceptions (Bradbury's distinctively poetic and dreamlike prose, for example). Tons of modern sci-fi authors write stories with more literary merit, better clarity, better-conveyed action, more believable characters and dialog, and so on. The improvements there are really obvious when reading, say, an early 70s The Hugo Winners or collections of 1950s stories, and then a 90s The New Hugo Winners.

Whether their ideas and worlds stand up to Asimov and company, or happen to be appealing to a particular reader, is another matter, but the command of writing itself is overall better, I'd say. I don't think a modern author would have much luck getting novels or stories in major magazines published with Asimov-tier dialog & characterization, for example.

Plus those eras were full of tons of garbage, in addition to what we remember. New Wave (60s and 70s) in particular tended to some very special interests that I don't think have held up too well, with some exceptions. A lot of that era's stories are very in love with sex & drugs and not in a way that really translates into a good tale. Most of those aren't exactly well-beloved these days.

On the flip side, there are definitely some modern sci-fi authors that are among the most famous but have some serious problems putting together an entirely good novel. I'll refrain from naming names but among them is maybe the only author whose book has ever made me mad at them for wasting such a good idea, setting, and opening, by just having no damn follow-through and getting super lazy in the last 1/3 or so. They're probably a top-5 sci fi name, among active authors today. But I stand by the overall quality level being notably higher on at least some dimensions, and it's even true for that guy.

fao_5 tahun yang lalu

> Whether their ideas and worlds stand up to Asimov and company, or happen to be appealing to a particular reader, is another matter, but the command of writing itself is overall better, I'd say.

But that's just the thing -- science fiction is about the ideas, and about the sociological and psychological impact of those ideas.

Whether a work has literary merit is neither here nor there for most people, and "science fiction" as a genre has been looked down upon by literary folks since it's inception. I don't know why anyone would be interested in praise from a community that is so invested in snobbery.

Regardless, science fiction authors of the time did have literary merit, if that's what you care about. LeGuin and Butler, are both regarded well in modern literary circles as far as I know. Vonnegut himself is regarded well also. At the time however, their books were condemned as being inadequate by the prevailing literary reviewers for various reasons unrelated to their actual writing.

Work from that time is perfectly functional and servicable for the most part, especially if you look at the places where the stories were submitted and published -- a small majority of Clarke's works ended up being published in Playboy, I don't think anyone can blame him for not creating a literary masterpiece when the majority of places that would actually pay him for his work were essentially pulp magazines.

shkkmo5 tahun yang lalu

We have a number of very good modern science fiction authors that easily rival them. While none of them may be as iconic, I think that has more to do with competition in the genre and breadth of the market.

omgwtfbbq5 tahun yang lalu

Name some? My experience with authors like Cixin Liu and some contemporaries is that they are unreadable, this could just be bad translations in Liu's case but I'm not at all convinced modern writers rival the classical greats in SciFi.

shkkmo5 tahun yang lalu

I found myself similarly underwhelmed by Cixin Liu given all the good things I'd heard.

I'm horrible at remembering names, but three of my favorites off the top of my head are Peter Watts, Neil Stephenson and Kim Stanley Robison.

I've generally had decent luck finding new authors by just by seeing who gets Hugo and Nebula nominations.

Sophistifunk5 tahun yang lalu

FWIW either the writing or translation seems to get better as 3BP progresses.

TomSwirly5 tahun yang lalu

> Asimov / Clarke / Lem

Asimov's SF is, sadly, hard to read these days. Clarke has held up better but still a bit dated. Lem reads as fresh as ever, but he's not so well known.

(What happened to Bradbury and Heinlein?)

Gene Wolfe is only one generation younger than these guys, and he just died, but I consider him a better writer than all the others I mentioned except Lem.

Sophistifunk5 tahun yang lalu

Peter F Hamilton. Start with Pandora's Star if you want more Asimovish, or Reality Dysfunction if you want it more Lemmy \m/.

FpUser5 tahun yang lalu

I read a lot including Sci-Fi and did not find any who are close. Does not mean they do not exist. Most likely not as famous and not exposed to wide public.

nitrogen5 tahun yang lalu

For a new generation of 10000 every day.

https://xkcd.com/1053/

[deleted]5 tahun yang lalu

giantg25 tahun yang lalu

Bleach works as surface decontamination agent. They know it works for CWD, and I expect it works for others too.

fao_5 tahun yang lalu

> Bleach works as surface decontamination agent. They know it works for CWD, and I expect it works for others too.

I remember reading that nothing but incineration would work in the context of Food-and-Mouth disease, but it seems modern techniques in brain surgery do use bleach. Interesting.

Essentially, the only thing that works is disintegrating the prions. You can't wash them off, since you're simply washing them into the water cycle. You can't deal with them in a way that preserves other biological matter, since they are proteins, disintegrating them means disintegrating any other proteins in that area.

iamthepieman5 tahun yang lalu

I'm missing the reference to foot and mouth disease. FMD is a viral infection and is not caused by prions.

fao_5 tahun yang lalu

IIRC at the time the BSE outbreak in the UK[1] was referred to as "foot and mouth disease" and I've heard people refer to it as that since then. I wasn't even aware they were separate things.

[1]: https://en.wikipedia.org/wiki/United_Kingdom_BSE_outbreak

shkkmo5 tahun yang lalu

There may have been some innacurate reporting, but FMD and BSE present very differently in humans so I doubt there was deep confusion.

sokoloff5 tahun yang lalu

For brain surgery, it seems like washing them off the implements and into the water cycle is still an enormous win in terms of risk.

fao_5 tahun yang lalu

I'm not sure you understand how irresponsible that is.

NoSorryCannot5 tahun yang lalu

Do you understand how irresponsible it is? It might be but it's obvious. How common are prions? How many prions get into the water cycle already? Prions probably don't go through the water cycle's gas phase. Are we worried about seafood? Surely lots of prions end up in oceans and lakes already.

It sounds a bit scary but it's not clear that it would change the risk profile.

sokoloff5 tahun yang lalu

That might be. I'm not an expert.

Is it more irresponsible than not removing them prior to operating on someone's brain?

fao_5 tahun yang lalu

You're acting like those are the only two options available, though. That's not the case.

ben_w5 tahun yang lalu

Foot-and-Mouth disease has a vaccine. I believe burning was for economic reasons: vaccination is expensive; the reproductive cycle (and by extension the population) is essentially human-controlled and the livestock population can recover quickly; and "we don't need to vaccinate" increases livestock sales.

markdown5 tahun yang lalu

Foot

Metacelsus5 tahun yang lalu

Gene-drive to edit wild animals so that they don't express PRNP (or express a stabilized version)

titanomachy5 tahun yang lalu

Stabilized maybe, it’s probably necessary for something even if we don’t know what yet.

adamhp5 tahun yang lalu

This really puts it into perspective...

senthil_rajasek5 tahun yang lalu

There is also a study from 2014 that suggests that prions were not detected in stems even though the roots of wheat plants were exposed to them. I don't know if there is conclusive evidence yet on getting sick by eating a plant based diet.

"This suggests wheat was unable to transport sufficient PrP(TSE) from the roots to the stem to be detectable by the methods employed."

Source: https://pubmed.ncbi.nlm.nih.gov/24509640/

medstrom5 tahun yang lalu

>It is thus possible that there is a progressively accumulating number of prions in the environment

I really need to grow my own food.

xeromal5 tahun yang lalu

Make sure to keep the deer out!

TearsInTheRain5 tahun yang lalu

Maybe this is a naive question, but your immune system works by identifying foreign proteins and killing those cells. Is there no way for your immune system to target cells contaminated with prions?

LorenPechtel5 tahun yang lalu

The fundamental issue with prions is that they are *not* foreign. Prions are legitimate proteins made by your own body but folded wrong. A misfolded protein acts like a catalyst to cause more misfolding. Prions are *not* alive, they have no ability to reproduce other than through misfolding existing proteins. It's basically a form of crystallization.

littlestymaar5 tahun yang lalu

Antibodies are shape-sensitive though, so there could be antibodies targeting the mis-folded protein. In fact, maybe many people exposed to them where able to develop an immune response and we just don't know about it because these people never had any symptoms (think about the survivor bias, but in reverse, or from the point of view of the prion).

codingbeer5 tahun yang lalu

Whenever a topic about something that could potentially destroy humanity comes up (say, climate issues), there is always some comment that makes a reasonable argument of why things are not as bad as they seem.

This never happens when prions are the topic.

dcolkitt5 tahun yang lalu

The simplest counterpoint to prion cataclysm fear is that it hasn't happened yet. That implies a low chance that it'll happen anytime in the near future.

There's no evidence that any significant human population has ever been wiped out by prions at any point in the history of the species. Heck, we haven't even observed widespread (>10% infected) prion disease in any species.

At least with something like climate change, there's the fact that post-industrial activity is doing something different than pre-industrial history. But this reasoning doesn't hold for prion cataclysm. It's more like asteroid apocalypse. Yes it could happen, but statistically it's not something we have to worry about on a civilizational timeframe.

I don't see any justification for why humanity in 2021 is courting prion disaster to a greater degree than it was in 2000 BC. Animal husbandry has existed for fifteen thousand years. Maybe globalization would spread a prion outbreak faster. But the point is we've never even observed localized prion collapse. If prions were that dangerous, there should already be regional areas that have transformed into total no-go zones.

XorNot5 tahun yang lalu

Conversely industrialized meat production is relatively recent, and so the volume of prion's being introduced into the environment is potentially much higher then the rate at which they are degrading naturally, not to mention the degree of regional cross-mixing of sources.

Prion contamination can now be shipped around the world in about 24 hours, where it can enter new populations much more easily.

csharptwdec195 tahun yang lalu

> regional cross-mixing of sources.

This is the part that worries me. If something happened like this even 300-400 years ago, it would probably just wipe out that village.

Amezarak5 tahun yang lalu

Proteins do eventually break down in the environment. If you hugely multiply the number of humans and population density, you are creating a situation where prion diseases can become endemic where they couldn't have been before.

This is probably exactly what happened with CWD - deer overpopulation plus the random protein misfold created the conditions for a contagious disease where it didn't exist before.

wombatpm5 tahun yang lalu

Or could it be that we have it and don't know it. Alzheimer cases are growing as we remove other causes of death. Dementia is just accepted as a consequence of growing old. It not like we section every brain of an old person who dies.

We only noticed it on our farm animals because we brought the cycle time down as we fed downer cows to other cows as a source of protein

TomSwirly5 tahun yang lalu

What's this "we"? Some of us aren't going to get CJD because we aren't implicated in this whole cow-eating activity.

Teever5 tahun yang lalu

> I don't see any justification for why humanity in 2021 is courting prion disaster to a greater degree than it was in 2000 BC.

While the kinds of people who would fly planes into buildings, release nerve toxins in subway stations or club entire villages of outsiders to death have always existed the ability to research and manufacture prions did not exist in 2000BC.

That combination of desire to destroy the world and the legitimate ability to do it with rapidly more accessible biotechnology is a disconcerting reality that we need to account for when discussing theses things.

Imagine a religious extremists organization mass producing prions and placing them in key parts of food supply chain. We probably wouldn't have a clue until it was too late.

littlestymaar5 tahun yang lalu

Biotech as a whole and its misuse by fanatics (or public servants in states with dubious moral …) is a massive concern for the 21th century, but I don't really think prions deserve a special place in this list. There's way too much scary things in here (gene drive, artificial viruses, intentional smallpox spreading, etc.)

Teever5 tahun yang lalu

That may be the case but it's a different issue than "Why are prions more of a threat to humanity than they were 4k years ago?"

With that said I sort of disagree with you. Prions are particularly attractive to a hypothetical bioterrorist due to the difficulty in detecting them, their durability and also the delay on effects. As I said above, once you know you have a problem it will be too late.

Keep in mind these are just the prions that nature has produced. It is quite possible that humans are able to modify existing threatening prions or develop whole new classes of threatening prions that haven't developed in nature.

Overall I think the most effective strike from a bioterrorist won't come from a single attack vector but a well timed combination of vectors. We've seen how something as minor as COVID has taken all of our attention and resources, now imagine if a terrorist timed something like COVID with a follow up strike like prions.

littlestymaar5 tahun yang lalu

> hypothetical bioterrorist due to the difficulty in detecting them, their durability and also the delay on effects.

Terrorism is about fear, and that comes with spectacular actions even if it kills relatively few people, not with stealthy poisoning of big population.

You're not gonna have anything remotely as impactful as 9/11 with prions, even if you killed a million people in the long run.

Is it a good weapon for a nation state going at war, or committing a genocide. Definitely yes! But it sucks for terrorism.

todd85 tahun yang lalu

> The simplest counterpoint to prion cataclysm fear is that it hasn't happened yet.

Well a prion disease (called Kuru) did affect the Fore people of New Guinea seriously in the 1950s and 1960s as I recall. Here is what Wikipedia says about the Fore people:

> Furthermore, Kuru predominantly affected women, as women more commonly partook in cannibalistic religious rituals. Because of this, there was a significant sex imbalance in Fore society.[14] By some accounts, this gender imbalance reached a 3:1 male to female ratio at its worst. This affected the family structure of the Fore, as it became commonplace for children to be raised and cared for only by their fathers.[1]

[1] https://en.m.wikipedia.org/wiki/Fore_people

Y_Y5 tahun yang lalu

Did you read about this in Guns, Germs, and Steel by any chance?

todd85 tahun yang lalu

I do have the book, it's quite interesting, but I read about Kuru while it was still thought to be caused by an infectious virus. My recollection is a bit vague, but I believe it was in Time or Newsweek during the 1960s.

It was a scary sounding disease so I remembered it when years later it was discovered to be caused by prions and reported in The New York Times.

cwkoss5 tahun yang lalu

If a significant human population was wiped out in history - how would we know it was prions?

NoImmatureAdHom5 tahun yang lalu

This is the right way to think about it. That said, a 1% infection rate that isn't at all a threat to the species given normal biological processes* will cause us to completely flip our shit. While we wouldn't be wiped out, everybody would be real upset for a long while. Look at COVID.

*Technology has given us things like nukes, so...could be harder to predict these days.

littlestymaar5 tahun yang lalu

Do we know how long prions have been there?

I'm not afraid of being hit by some asteroid hanging there, but if one day we spotted an asteroid whose trajectory was straight to earth, then being terrified would be quite legitimate.

[deleted]5 tahun yang lalu

dennis_jeeves5 tahun yang lalu

>I don't see any justification for why humanity in 2021 is courting prion disaster to a greater degree than it was in 2000 BC.

Because the 'scientific' community need to keep their jobs. You know they too have mortgages to pay...

So... point being there is endless stream of fear mongering (some of which ends up being true) to justify their existence.

giantg25 tahun yang lalu

Actually, there is some good news. Many types of prions diseases do not spread from animals to humans (so far). There have been some studies around this with CWD, but also Scapie has been around for hundreds or even thousands of years and never made the jump. Even in areas with CWD, we see that herd numbers do typically rebound. It's believed that there is some genetic factor that prevents the onset of the symptoms, like the body's proteins are less likely to fold in that way, so you only accumulate what you eat, or that the body can clean up the misfolded proteins. So it's not like it would wipe out everyone. I think I remember reading that some people are also researching prion treatments with some limited sucess. I would imagine they would come up with something even more quickly if there was a epidemic type of situation, similar to how medical researchers around the world focused on covid.

throwaway3169435 tahun yang lalu

Not to be a doomer but there are scenarios where neither genetic variation nor research would solve the problem. The correct mix of virality, incubation and lethality would do it. Pneumonic plague is terrifyingly close. I wish there was a bigger interest and investment in biology, I think it’s the only viable path forward for humanity.

giantg25 tahun yang lalu

I was just discussing prions. Other pathogens do seem to present a bigger risk. I don't think anything would have 100% kill rate. Even weaponized microbes have like 99.8% or something. That still leaves about 16 million people. Plus there's all those preppers with their bunkers, some uncontacted tribes, people who live in isolated areas. Then it becomes more of a matter of how to organize, do they have the knowledge to survive in those types of conditions, etc. The species will survive, but it will be a very different world.

piker5 tahun yang lalu

Actually there is a positive anecdote often cited. The Fore people of New Guinea engaged in ritual cannibalism which lead to the propagation of prions across generations. When they stopped, the generations of disease also stopped.

https://en.wikipedia.org/wiki/Kuru_(disease)

joshuaissac5 tahun yang lalu

The Fora people even developed a prion that confers resistance to kuru:

> researchers [...] discovered a naturally occurring variant of a prion protein in a population from Papua New Guinea that confers strong resistance to kuru

> This community [...] has developed their own biologically unique response [...] this genetic evolution has happened in a matter of decades

medstrom5 tahun yang lalu

You jinxed it, so I'll be the "some comment": prions won't spread everywhere all at once, so humanity will probably self-isolate in at least a few prion-free clusters.

userulluipeste5 tahun yang lalu

Prion caused disease is indeed terrifying on individual level but, from its impact over the species' survival, it isn't that menacing. The decay takes decades, which is enough for breeding the next generation. Human life expectancy may get shorter, which sucks, of course, but humans lived shorter lives for most of their history. So no, it's not that bad. Even assuming that the entire human population will get infected from young age with no way to avoid it, it won't be that bad. It would come with a bit of adjustment, like having to raise kids sooner because the luxury to postpone (for life enjoyment or other pursuits) won't be there, but things will go on.

marcosdumay5 tahun yang lalu

Well, prion diseases are very rare in humans (or at least their transmission is rare), and there is no evolutive pressure to create a super-prion that can infect more people, like happens with other pathogens. Thus, the most likely situation is that it will continue to be very rare until we are able to change it into virtually non-existent by technology.

They are a really scary thing, but it's a meteor strike style of scary, nobody really expects it to happen before we can deal with it.

[deleted]5 tahun yang lalu

jeswin5 tahun yang lalu

I lost someone very close to me to sporadic CJD. This was some time back, but the episode is one of the most troubling moments in my life because of the suffering it inflicts on the patient - the hallucinations with no way to communicate or to stop it.

jeffkeen5 tahun yang lalu

I lost my mom this way in 2016 and nothing has made me wish for legal euthanasia more than witnessing that suffering and decline.

hyko5 tahun yang lalu

There is ongoing surveillance from the National CJD Research & Surveillance Unit: https://www.cjd.ed.ac.uk/sites/default/files/figs.pdf

one2three45 tahun yang lalu

I skimmed through it but my understanding is that this is not a prion outbreak. At least the relevant tests come back negative. Did I get it wrong?

NoGravitas5 tahun yang lalu

It's not a known prion, like CJD. It could still possibly be an unknown prion. Or any number of other things.

thehappypm5 tahun yang lalu

Prions cannot evolve, which is why they represent such a tiny space in diseases. They're really more a quirk of chemistry than biology.

throwaway3169435 tahun yang lalu

They can’t evolve independently but biological systems that produce proteins can evolve new proteins and new ways of manipulating proteins. A nightmare scenario would be a bacterial or viral mutation that produced a deadly prion.

thehappypm5 tahun yang lalu

This feels like being worried about sharks evolving machine guns.

robbiep5 tahun yang lalu

A nightmare scenario for me is the creation of mRNA coding for a misfolded PRNP being weaponised.

thehappypm5 tahun yang lalu

I don't think you need to worry too much about that, honestly. as far as bioweapons go mRNA is pretty involved.. if you need to inject your victim, that's already way too complicated. bioweapons are much more oriented towards tainting water supplies or air.

robbiep5 tahun yang lalu

I know a huge amount about the level of technical skill required to do something with mRNA. It’s involved today.

But in many ways it’s less technically involved than (re)creating the horse pox vaccine from scratch.

And in 20 years it could be as simple as something aerosolised (there are already research projects for nasal mRNA vaccines) we don’t know quite yet where this will all lead to - PRNP is small enough at 200-odd amino acids long that it can be easily encoded on mRNA and as a fucked up disease vector that’s surely worse than anything viral - a timebomb sitting in everyone’s bloodstreams waiting 5-50 years to go off

eganp5 tahun yang lalu

This probably isn't possible as the folding of PrP is not encoded in the mRNA of PRNP. The fold of PrP is a post-translational phenomenon, much like its glycosylation. This is true for many mammalian proteins, especially cell-surface proteins.

robbiep5 tahun yang lalu

I’m aware of that. However the specific choice of amino acids can heavily influence the propensity to fold in a particular way. For example homozygotes for M129 have a much faster rate of vCJD development. Since Prion Diseases occur sporadically, the rate of mis folding can be increased and it probably wouldn’t be that difficult (even computationally) to determine how you can get it to misfold at a much higher rate.

I’m not sure what you mean by the fold being post translational, all folding is post translational, it’s literally the protein curling up into the lowest energy conformation as soon as it encounters the cellular environment, with assistance from HSPs if necessary. Unless you meant something else?

orblivion5 tahun yang lalu

I'm not sure why this is the case. They replicate. There might be an error in replication, no? Or is that just really hard? Is it that their existence is in a sweet spot, and nothing even marginally different would work the same way?

arisAlexis5 tahun yang lalu

What happens if for example nestle has a contaminated factory product that millions eat and we will know it in 10 years from now?

phendrenad25 tahun yang lalu

Related to Havana Syndrome?

r7215 tahun yang lalu

From the article:

"Despite patients showing no trace of any known prion diseases, it hasn't been completely ruled out as a cause, he says.

Another theory is chronic exposure to what's called an "excitotoxin" like domoic acid, which was linked to a 1987 food poisoning incident from mussels contaminated with the toxin from the nearby province of Prince Edward Island.

...

Dr Cashman says they are also looking at another toxin - beta-methylamino-L-alanine (BMAA) - which has been implicated as an environmental risk in the development of diseases like Alzheimer's and Parkinson's.

BMAA is produced by cyanobacteria, commonly known as blue-green algae."

dmix5 tahun yang lalu

(3)

londons_explore5 tahun yang lalu

I wonder how common diseases like this are that impact just one or just a handful of people?

andai5 tahun yang lalu

The long tail...

londons_explore5 tahun yang lalu

Indeed. You frequently hear "Everyone is different", "Symptoms can vary" and "Everyone acts differently to XYZ treatment".

But perhaps these are all symptoms of diseases and combinations of diseases varying, rather than the people?

Brakenshire5 tahun yang lalu

“ Although rare diseases may be individually rare, they are collectively common, with 1 in 17 people being affected by a rare disease at some point in their lives. ”

https://www.gov.uk/government/publications/uk-rare-diseases-...

jokoon5 tahun yang lalu

I was scared for a bit.

This reminds me that I first read about early cases of covid around october or november 2019.

drran5 tahun yang lalu

How and where you were able to read this?

jokoon5 tahun yang lalu

It made some news, it was suspicious cases of pneumonia in china. First time I read the article, I was like "uh oh". It did not make the headlines though, but it was still noticeable. I think I spotted it on reddit or hackernews. Maybe the article was on BBC? BBC has quite a good world coverage.

I guess you can google news by year. I'm very certain those articles exist. Next time I heard about it, it was getting much worse.

samirelanduk5 tahun yang lalu

I don't think there were any reports online until December 31st.

herbst5 tahun yang lalu

I found one (english) Timeline that Starts before 31.

https://bfpg.co.uk/2020/04/covid-19-timeline/

I too remember talking about it on Christmas.

hattmall5 tahun yang lalu

I remember hearing about it prior to that as well. I remember because I was building a shed and had someone helping me who was talking about it and it was prior to the new year, early December at the latest. He heard about it on a Doomsday type podcast.

carlmr5 tahun yang lalu

According to Giuseppe Remuzzi there were strange pneumonia cases in northern Italy in November 2019, but as far as I can see they were not reported until March 2020.

Apart from that I remember the first reports from China in December 2019.

asenna5 tahun yang lalu

I remember reading about it end of December 2019. I believe it was a Reddit thread with some worrying reports on the ground.

awd5 tahun yang lalu

Funnily enough, there were some memes about it before December 17th 2019. Comparing some disease from 1920 with 2020.

satysin5 tahun yang lalu

I journal daily and like to capture the news among my daily record. The first time I found COVID/Coronavirus mentioned by BBC News was January 3rd 2020 https://www.bbc.com/news/world-asia-china-50984025.

It is of course possible I missed a story but before January 3rd there was little to no mainstream news coverage. It is quite frustrating to find accurate articles now as many sites are falsely returned in date range Google searches. All "mystery flu-like pneumonia" stories I've found reported in December 2019 are medical news so it is extremely unlikely someone would come across such an article as part of normal news reading.

phyalow5 tahun yang lalu

On January the 4th their were posters (printed A4) up on Public Transport in Hong Kong. I remember thinking it was very strange - I had heard a story on the BBC world service the night before. The posters warned of a "Novel Pneumonia from Southern China". How the world changed...

satysin5 tahun yang lalu

How it changed indeed! Reading my journal for the first four months of 2020 is fascinating. I must have read over those first few months ten times since. So much has happened with advice and guidance changing as we learn more about the virus and the disease. So much mis- and disinformation. Crazy behaviours all over. I am so glad I keep a journal as it will be quite something to read back in a decade or two.

Especially for my 8 year old son. He is old enough to understand things are not normal but young enough to not fully understand the gravity of the situation or have memories of much outside of his little bubble.

M2Ys4U5 tahun yang lalu

The BBC were definitely covering it in late December 2019 - I distinctly remember the TV coverage on New Years Eve.

satysin5 tahun yang lalu

If you have any links to articles or news reports I would be very grateful.

s1artibartfast5 tahun yang lalu

Here is a western news report from mid November: https://foreignpolicy.com/2019/11/16/china-bubonic-plague-ou...

dqv5 tahun yang lalu

If you can get access to an archive of South China Morning Post that hasn't been scrubbed, the term you want to search for is "mystery pneumonia" or "sars like".

satysin5 tahun yang lalu

Thanks, I have several Chinese/Asia news sources from December however I was asking for a BBC reference as M2Ys4U mentioned it was reported by the BBC on New Years Eve and the earliest I have is January 3rd.

dboreham5 tahun yang lalu

btw this is where the "19" comes from.

oblio5 tahun yang lalu

I heard about Covid in January. It was still kind of localized, at the time, we didn't know much about it.

Around mid-February I saw in the news that there was a somewhat big cluster in Italy and realized that everyone was screwed :-(

ekianjo5 tahun yang lalu

The first news about COVID were at the very end of december 2019 at least on mainstream media.

oblio5 tahun yang lalu

True, but between actually seeing the first news and figuring out: "Oh my God, this is going to be a global pandemic!", it took me a while. I think most people were in the same boat.

cmrdporcupine5 tahun yang lalu

My wife had taken a couple infectious diseases courses as part of her anthropology degree so she was tuned into this whole thing and the first article she read about it end of December I remember her freaking out. "Oh, great, another SARS-type virus has been found, we're screwed" and me being like "honey, you're being paranoid. relax."

I hate it when she's right.

Spare_account5 tahun yang lalu

https://www.google.com/search?q=suspicious+cases+of+pneumoni...

This URL searches Google for your phrase "suspicious cases of pneumonia in china" filtered for results between Oct 1 and Nov 30 2019

Leherenn5 tahun yang lalu

Some months back, I had the same recollection: an article about suspicious pneumonia cases in China published by the BBC sometimes in December. I did some searching, and nothing, I think the first article is from the 31st of December. Funny how the mind can trick you, maybe it is linked to "Déjà vu" [1].

[1] https://en.wikipedia.org/wiki/D%C3%A9j%C3%A0_vu

TorKlingberg5 tahun yang lalu

Unless you have a link to the article from that time I'm going to guess that is a false memory or something unrelated.

Spare_account5 tahun yang lalu

Not the same guy, but perhaps you can help me understand if this event is related?

https://www.nytimes.com/2019/11/13/world/asia/plague-china-p...

Date: November 13 2019

>Li Jifeng, a doctor at Beijing Chaoyang Hospital where the two people sought treatment, wrote on WeChat, a social media platform, that the patients sought treatment on Nov. 3

>“After so many years of specialist training, I’m familiar with the diagnosis and treatment of most respiratory diseases,” wrote Dr. Li. “But this time, I looked and looked at it. I couldn’t guess what pathogen caused this pneumonia. I only knew it was rare.”

tremon5 tahun yang lalu

At most it might have been a precursor to SARS-Cov-2, but it's unlikely to be the same agent. We've seen how wildly virulent that variant is; there's no way I can see that that virus could have been found in the wild in a city like Beijing and not cause the same problems we saw in Wuhan two months later.

dboreham5 tahun yang lalu

This makes sense if the disease propagates linearly like bacteria on a petri dish. But it probably doesn't. It may spread more like a wildfire -- there can be burning branches blown miles from the main fire that only smolder or set a small patch of grass burning which flames out due to lack of fuel. Meanwhile the main fire is only stopped by water or other major geographic feature.

giantg25 tahun yang lalu

I could see it as possible. The spread is asymmetric. You could have one infected person who is not a super spreader only infect a few other people who just happen to be asymptomatic and not spread it to others. When your overall infected population is very small, like in the beginning of a jump from animals to humans, then you have a chance to get lucky in this way. Once you get to a larger infected population (like dozens of people) then you're more likely to have a super spreader and less likely to get lucky with only contacting asymptomatic people.

saiya-jin5 tahun yang lalu

There are back-confirmed cases of covid in Italy in September 2019, so the Wuhan incident is definitely not a patient-0 situation (even if it could be a ground-0 location from previous patient-0 situation, but its just a guess and we'll never know).

Given that, having a case in Beijing in November 2019 is entirely plausible

tremon5 tahun yang lalu

Following that train of thought, what changed between Italy/Beijing and Wuhan? Was it sheer luck that made the first major outbreak happen in Wuhan, and not in Milan or Beijing? Or Los Angeles for that matter, I think I heard reports of suspect pneumonia cases from the US as well?

What I was alluding to with "precursor" was that it may have been the same virus, but that some mutation in Wuhan might have made it so much easier to spread. My mind just has trouble imagining a model where a virus can lie pretty much dormant for half a year in different populations, and then surge in one place only and spread from there. That may well be a limitation of my mind, I simply don't see how that would work.

dannyw5 tahun yang lalu

If you have a deck with 2 hearts and 10 spades, your first flip could very well be a heart, even if with enough flips, 10/12 are spades.

What I mean by this is that viruses may have arbitrary behaviour when the infected population is small. Let's say as of September 2019, five people in the world were infected with COVID. It's actually not improbable or crazy that the epidemic outbreak only started in Dec/Jan.

callamdelaney5 tahun yang lalu

I'm pretty sure I read a very accurate post on /r/wallstreetbets about covid-19 in late 2019/early 2020.

paunstefan5 tahun yang lalu

I remember someone made a joke about how there were pandemics in 1920, 1820 and 1720, and another user commented that there is a weird flu in some Chinese city. This was either in the last week of 2019 or very early 2020.

dqv5 tahun yang lalu

Yes I remember a friend who lives in Hong Kong posting a news article (from a Chinese news source) describing a "mystery pneumonia" the first or second week of December 2019. He hypothesized that it was because of bushmeat. I think the news article got scrubbed. I wish I had saved it.

[deleted]5 tahun yang lalu

hobbescotch5 tahun yang lalu

The province of New Brunswick has set up a website tracking this here: https://www2.gnb.ca/content/gnb/en/departments/ocmoh/cdc/neu...

cpncrunch5 tahun yang lalu

Interesting. The symptoms they list are entirely typical of FND, which accounts more than 1 in 7 referrals to a neurology department and is at least as common as MS [1]. They list 48 active cases, but there would presumably be a lot more FND cases than that in NB. Also, it seems they only uncovered this new syndrome when they actively started looking for them via the Creutzfeldt-Jakob Disease Surveillance System. Could it just be that many doctors and neurologists are unaware of FND, even though it is by far the most common neurological disorder they see?

[1] http://www.rcpe.ac.uk/journal/issue/journal_41_1/stone.pdf

rvz5 tahun yang lalu

Not this again. I really hope that the incubation period is not around the corner with another wave of vCJD 'variant Creutzfeldt-Jakob disease' aka (Mad cow disease).

forgotmypw175 tahun yang lalu

As mentioned in previous submissions, because it is happening in a certain area, industrial pollution can be considered as a possible cause as well.

wobblyasp5 tahun yang lalu

That was the going assumption as far as I understood (I'm from the area). They're hasn't been direct health advise on areas to avoid either, so it's slightly disconcerting.

throwaway3169435 tahun yang lalu

Bathurst and Moncton are far enough apart that I’d rule that out.

giantg25 tahun yang lalu

I would generally agree, except in wonder what the aquifers/groundwater looks like in that region and also the sea water along that coast (likely more of a biological source than an industrial one in that case, I think).

throwaway3169435 tahun yang lalu

My bet is on either bacterial or agricultural source contaminating well water.

forgotmypw175 tahun yang lalu

Why do you think that, and what do you mean by "agricultural source"?

throwaway3169435 tahun yang lalu

Well water contamination is common and people are increasingly neglecting to test on a regular schedule. One of the symptoms listed is gastrointestinal upset which makes me think it could be something that was ingested. They mention that Roger’s condition has stabilized now that he’s in a care home, tenuous but it seems location change resulted in change in progression of symptoms. By agricultural I mean waste from livestock.

forgotmypw175 tahun yang lalu

Which of these is isolated to agriculture pollution, but not industrial pollution?

withinboredom5 tahun yang lalu

Since prions can be inhaled via aerosoles[1], I often wonder how long it will be until vaping causes prion diseases to rapidly spread. Or new prions to be introduced.

[1]: https://journals.plos.org/plospathogens/article?id=10.1371/j...

sirsinsalot5 tahun yang lalu

What on earth does vaping specifically have to do with the aerosol transmission of prions? Unless there are misfolded proteins in the liquid, but that's just as likely as them being in your beef, the air around you, someone chopping meat or anything else.

Your vaping reference seems odd.

withinboredom5 tahun yang lalu

If (and it's a big if) they were in the liquid, someone walking down the street and hitting their vape could "infect" quite a lot of people. The delay between that point and the onset of symptoms would make it very hard to pinpoint the vape as the cause.

It's just interesting to think about.

shoemakersteve5 tahun yang lalu

> It's just interesting to think about.

Is it? Your line of thinking is really confusing to me. The likelihood of this happening by accident is effectively 0%. Even if it was intentional, this makes even less sense. Because then the person would not only have to find some prions to put in their vape, they would also have to inhale it themselves to then blow it into other people's faces?

I just really don't understand where you're coming from or why you would say any of this.

Cthulhu_5 tahun yang lalu

Eh? Loads of bad stuff can be inhaled via aerosols so idk why you're mentioning prion diseases specifically. I mean not that long ago there was a lot of 'off the market' e-liquids that would cause popcorn lung.

Sunspark5 tahun yang lalu

Lots of aerosol "air fresheners" out there in operation too, that use petroleum gas as carriers. It's just accepted that it's fine to inhale petroleum gas.

peteretep5 tahun yang lalu

> Several patients have presented with transient "Capgras delusion", a psychiatric disorder in which a person believes someone close to them has been replaced by an impostor.

> "It's quite disturbing because, for instance, a patient would tell his wife: 'Sorry ma'am you cannot get in bed, I'm a married man' and even if the wife gives her name, he'd say: 'You're not the real one,'" Dr Marrero says.

You could spice up a lot of marriages if you could harmlessly cause Capgras delusion for a few hours

medstrom5 tahun yang lalu

As long as you induce it consensually, right? It sure would be uncomplicated, no real third party needed.

regularfry5 tahun yang lalu

That... wouldn't work. Consent must be continually given, and for Capgras to work you can't be aware it's a delusion at the time you're experiencing it.

thatguy09005 tahun yang lalu

I think the idea is that if you wanted to spice up a marriage with an affair, you could use this to "cheat" on your spouse with your spouse. Both parties would be consenting the whole time. One would just think they are cheating.

regularfry5 tahun yang lalu

Oh, I understand the concept. It's broken. If I am "consenting", but not to the action that's actually happening, I am not, in fact, consenting. If I believe that I am consenting to sex with someone that's not my husband, in that moment I am not consenting to sex with my husband. If they then have sex with me, knowing that that is my state of mind, that is non-consensual.

peteretep5 tahun yang lalu

You do not understand the concept:

I decide I want to try a bit of "something new", and I pay someone to induce a Capgras delusion in me. At which point, my wife seems like an imposter. I wish to have sex with this new person, and I am also sure my real wife has given me permission to, prior to this. You can tell me as much as you like that it's "really" my wife, but, in my delusion, I am quite sure it's just someone who looks like her, and delightfully and consentfully have sex with the imposter new person -- who is giving their consent the whole time -- with all the thrill that entails. Afterwards, the delusion wears off, and I realise it was just sex with my wife. Everyone has consented at every point to this.

regularfry5 tahun yang lalu

I do understand the concept. You have not changed my understanding with this description.

If I am suffering a delusion, and you know that, then you know that I am not mentally competent. You know that, if I agree to something, I do not correctly understand what I am agreeing to. Any expression of consent you extract from me is invalid.

thatguy09005 tahun yang lalu

This seems extreme. You are consenting to be deluded, knowing everything that will come afterwards. And the consenting during as well

regularfry5 tahun yang lalu

But you don't know everything that will come afterwards. You know what's planned.

The consent during cannot be valid. It's not informed: in fact it's specifically misled, and that is known to the other participant.

thatguy09005 tahun yang lalu

So you are saying there is no way for two willing, informed adults to consent to this happening?

regularfry5 tahun yang lalu

Yes. That is what I'm saying. If consent needs to both informed and continuous, it's not possible. It's fundamentally broken.

peteretep5 tahun yang lalu

Yes, that’s why you’d get consent before hand

regularfry5 tahun yang lalu

Consent beforehand doesn't bind or imply consent during the act. If you're saying consent beforehand counts, you're saying that it's not possible for anything to happen that would cause me to change my mind if I was in a mentally competent state.

medstrom5 tahun yang lalu

This discussion boils down to if consent must be continually given or if you can give it ahead of time. And I'm not up to date, but I believe it's not a settled discussion even in academia. The problem of continual consent is that if it must be continually given then you can never give your partner permission to initiate sex with you before you wake up, or agree that it's fine to have sex while drunk, and these are things a nontrivial amount of couples want and are not harmed by, which means such a rule is inadequate.

andy_ppp5 tahun yang lalu

I do wonder if it’s possible that vaccines that are designed to make the body produce certain parts of the spike protein could lead to prion diseases accidentally. Are we sure of the proteins coded for and also that this coding can’t produce misfolded proteins? It’s pretty unlikely but also we don’t have long term testing of these proteins in the body.

* please note I’ve had the first dose of Astra Zeneca and intend to get the second.

devit5 tahun yang lalu

Seems like prions only "infect" the same kind of protein, so a misfolded spike protein that the vaccine might produce would only cause other spike proteins to misfold, and they almost surely don't occur naturally in human tissue so there should be no risk at all.

andy_ppp5 tahun yang lalu

Excellent explanation I guess the short term effects of producing these proteins is pretty small, that’s good to know.

anonymouse0085 tahun yang lalu

As I commented below, I have only drawn loose correlations with no direct evidence as well... so to hear the mechanics of prion misfiling other same proteins is much appreciated.

wobblyasp5 tahun yang lalu

I'm unsure of how vaccines are related here. First case was in 2015, and cases are relatively contained to an area

anonymouse0085 tahun yang lalu

I’m wondering if the original Covid infection runs the risk for latent prion issues too —

There’s just no reason for the mass hysteria from governments from what I’ve seen spread amongst my friends - and I run dangerously close to leaving the “anecdotal data gravity well” with how many observations I’ve tracked, deaths included.

It felt like they were withholding some long term effects to keep everyone calm.

[deleted]5 tahun yang lalu

tsimionescu5 tahun yang lalu

More people dead of Covid than TBC is not enough in your mind to make it a serious risk? How can you jump to prions (which no other viral disease has ever been known to cause) before simply looking at the massive death toll, despite the unprecedented restrictions?

anonymouse0085 tahun yang lalu

Perhaps I’m a bit too relative in thinking our threshold for responding to pandemics should be in proportion to the response than previously seen pandemics and events... so here’s what I mean:

“Any mortality comparisons between these two pandemics in the United States, 2020 and 1918, must differentiate between totals and rates. The current US population, a little more than 330 million, is more than three times larger than the population in 1918, estimated at 105 million. The 675,000 deaths attributed to the influenza epidemic made up 0.64 percent of the total population, a little more than six in every thousand people. By contrast, the more than 500,000 deaths attributed to COVID-19 make up about 0.15 percent of the total population, or between one and two in every thousand people. If COVID-19 caused deaths at the same rate as the 1918 epidemic, the total would approach two million. Even the disturbing projections of more than to 600,000 deaths by July 1, 2021, would still remain below the rates recorded in the earlier epidemic.“

https://www.healthaffairs.org/doi/10.1377/hblog20210329.5129...

So, by one token, you could say we did better through the response of drastic measures...

Or we could note that the world didn’t perform wide scale testing in the 1918-1919 pandemic to determine how many other seemingly unrelated deaths also contained Spanish Flu, or how many pandemic cases they really had back then.

So without knowing how many contracted both sets of viruses, it’s tough to make a call on the R0, but could a rough approximation of a pandemic's severity could be the proportion of populations, strata by types (age, whatever you’d like), that experienced fatalities?

If so, does that mean the flu then was worse? What was the world response at that time? I’ll bore you less and say that the measures taken then were no where near as strict as those now.

So comparing that response to this new worlds response... why does there appear to be a disproportionate response that appears more strict in this age than the last?

Many possible answers can fit the set of facts above... one extremely unlikely answer I’ve entertained is that the Covid-19 infection is latent in nature and could cause issues down the line.

Prions share in that characteristic in some research... as in they have a latent emergence that some have been thought to be misdiagnosed as dementia or the like, when in fact it is probably mad cow disease.

So those super, super loose correlations I look at and say, “hmm if someone tells me Covid causes a latent prion disease with solid facts, I wouldn’t be surprised” — I’m mot advocating that is what’s happening, I’m just saying ruling it out as completely invalid without evidence is a little short sighted.

Fauntleroy5 tahun yang lalu

India is currently at 3.5k deaths _per day_. Anyone who's been paying attention knows that the issue at hand isn't that COVID is some kind of super-disease, but that it causes a strain on emergency medical resources that can quickly cause chaos. Ventilator resources used up, oxygen unavailable, etc.

There's no need to come up with a new conspiracy theory about COVID every day. The facts are way more boring, but it all does ultimately check out.

anonymouse0085 tahun yang lalu

Right on - I would just say fairly early on in this process we knew what Covid did when to who and how, but we didn't adjust the strategy in the US to match the new information. Which left a big gaping 'Why'? (and yes, easily filled in with p < 0.05 possibilities)

tsimionescu5 tahun yang lalu

Why would you compare the death rates from a pandemic that happened immediately following a devastating world war, with famine and poverty all across the globe, at the very beginning of real medicine, in a world of borders; with one happening in 2020 in a globalized world where we have access to much more advanced medical knowledge, and where an outbreak in Wuhan can become an epidemic in New York?

Not to mention, the initial death tolls in the unprepared places, like Lombardia in Italy or Iran, should be motivation enough for a quick and decisive response. Unfortunately, neither the US nor Europe nor Russia were smart enough to take such an approach, and instead of a quick and thorough lockdown + contact tracing that would have hurt daily life for maybe 2 months before back to normal, with almost no casualties (see Vietnam to note that this was entirely possible), we are getting 2 years of lukewarm responses that leave the same order of magnitude of deaths as the 1918 pandemic.

anonymouse0085 tahun yang lalu

That’s a thoughtful discussion, and I hear your points, I’m just keeping a bit of “open weights” to the possibilities.

To your point, we are more medically advanced and can track cell phones across the globe, which in some ideas could mean we need to respond less in an aggressive amputation of economies and more focused proper risk assessments - which also means I would have advocated from the beginning to ground all travel (air esp) and any economic activity to assess the situation for a month or two.

So when evaluating Italy or Iran, we should be doing genetic testing and massive analysis on who gets severe vs mild cases, and is is a heritable trait that could explain anomalies in various communities.

Again, beautiful discussion... not saying you’re wrong, just leaving options open.

williesleg5 tahun yang lalu

anonuser25 tahun yang lalu

(2)

callamdelaney5 tahun yang lalu

There was some research which suggested that a not insignificant number of dementia cases were actually misdiagnosed mad cow disease. Because CJD can stick around for a while without taking affect, I wouldn't be surprised.

throwaway3169435 tahun yang lalu

The age range starts at 18 for this outbreak.

HDMI_Cable5 tahun yang lalu

I'm not a neurologist but maybe some of the prions from Mad Cow Disease can further the progression of dementia?

psyc5 tahun yang lalu

This is a running gag for William Shatner’s character in Boston Legal.

tcbawo5 tahun yang lalu

I know someone in their early 40s experiencing dementia-type symptoms (consistent with frontotemporal dementia). It is early but is quickly getting worse. It's heartbreaking to see how this is affecting his spouse and children. So far, they don't have a diagnosis. They are still running tests. It has to be quite frustrating not to be able to point to a root cause. So little can be known without direct access to the brain.

nick_5 tahun yang lalu

I have read a couple of times that an HSV infection (super common) can get into the brain and cause dementia. Just trying to help here, but maybe they could try an HSV antiviral?

GioM5 tahun yang lalu

The forests of New Brunswick are routinely sprayed with large amounts of glyphosphate herbicide.

Is it possible that this is the cause, instead of prions?

[1] https://www.conservationcouncil.ca/where-our-forest-is-being...

[2] https://www.cbc.ca/news/canada/new-brunswick/liberals-greens...

[3] https://nben.ca/en/component/tags/tag/glyphosate-ban

[4] http://www.stopsprayingnb.ca/?page_id=118

bluGill5 tahun yang lalu

No, glyphosphate is used in a lot of different places and is well studied. There are the usual conspiracy theories but the science is pretty strong.

rubicon335 tahun yang lalu

What science are you referring to? The most I've heard about it is the Bayer lawsuit.

"A San Francisco jury initially awarded Johnson $289.2 million after finding the chemical glyphosate in Roundup caused his non-Hodgkin’s lymphoma" [1]

[1] https://www.reuters.com/article/bayer-glyphosate-lawsuit/bay...

bluGill5 tahun yang lalu

A jury trial is not science. Juries tend to be swayed by emotions and sticking it go big companies not right. (I can't offer you anything better to replace them though )

There have been many real scientific studies done. https://www.epa.gov/pesticides/epa-finalizes-glyphosate-miti... is the most readable summary

rubicon335 tahun yang lalu

A publication from the EPA citing science, without linking the studies, is also not science.

I honestly do not trust the EPA to do a fair review here, given how deep the connections between big business and government are. It's extremely naïve to assume this was a completely unbiased, purely scientific undertaking.

Your point is fair too, that juries tend to be swayed by emotion. Unfortunately, unless a true third party does an actual scientific review, I'll be avoiding the stuff.

decasteve5 tahun yang lalu

A direct causal relationship is unlikely, however, the link between increased cyanobacteria in our waters and glyphosate spraying is possible and being studied elsewhere.

New Brunswick also carried on with heavy spraying of DDT into the 1980s stopping much later than most places.

There have been more diseased moose sightings in recent years too.

What about the possibility of contaminated seafood?

So many possibilities. Quite scary with no concrete answers.

anthony_romeo5 tahun yang lalu

In other Prion news, there's a prion disease, Chronic Wasting Disease (CWD), affecting populations of wild Deer, Elk, etc. in North America and other chunks of the world. It's not yet known to infect people, but please be careful when eating wild game. [1]

My mind first went to this issue when I read the headline, though upon reading the article it's probably something else, as CWD isn't really a thing in that region [it's more of a Mountain state kind of thing thing]. And, of course, I'm not a prion expert, so I only vaguely know what I'm writing about.

[1] https://www.canada.ca/en/health-canada/services/food-nutriti...

eganp5 tahun yang lalu

The species barrier in prion transmission appear very strong [0]. Transmitting a prion from a hamster into a mouse often takes multiple passages of direct in brain inoculation. In these closely related species, perhaps it's less strong. It's doubtful that CWD can transmit to humans at typical exposure levels. In vitro, conversion of human PrP is possible, but exceedingly difficult.

[0] https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2736662/ [1] https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5667731/

loonster5 tahun yang lalu

CWD is in 25 states now. Some of them have cattle.

https://www.cdc.gov/prions/cwd/occurrence.html

admash5 tahun yang lalu

Compounding USDA's lax practices has been its refusal to allow beef processors to independently test cattle for mad cow disease. In 2004, Creekstone Farms, a Kansas processor of black Angus beef with a large Japanese clientele, asked for permission to test its 300,000 cattle for BSE using a $500,000 testing site it had built to USDA specifications.

But the agency ruled that the BSE test was licensed only for "surveillance" of animal health, and rejected Creekstone's request because it implied "a consumer safety aspect" that was "not scientifically warranted."

https://www.eurekalert.org/pub_releases/2006-05/uoia-ftf0515...

mannerheim5 tahun yang lalu

This is an incomplete description of the USDA's rationale. Although I agree that there shouldn't be a ban on private testing, the USDA's position is that BSE testing is ineffective at the time most cows are slaughtered, and would therefore provide an unwarranted impression of safety.

> NOT A FOOD SAFETY TEST

> BSE tests are not conducted on cuts of meat, but involve taking samples from the brain of a dead animal to see if the infectious agent is present. We know that the earliest point at which current tests can accurately detect BSE is 2-to-3 months before the animal begins to show symptoms. The time between initial infection and the appearance of symptoms is about 5 years. Since most cattle that go to slaughter in the United States are both young and clinically normal, testing all slaughter cattle for BSE might offer misleading assurances of safety to the public.

> ...

> Why doesn't USDA test every animal at slaughter?

> There is currently no test to detect the disease in a live animal. BSE is confirmed by taking samples from the brain of an animal and testing to see if the infectious agent - the abnormal form of the prion protein - is present. The earliest point at which current tests can accurately detect BSE is 2 to 3 months before the animal begins to show symptoms, and the time between initial infection and the appearance of symptoms is about 5 years. Therefore, there is a long period of time during which current tests would not be able to detect the disease in an infected animal.

> Since most cattle are slaughtered in the United States at a young age, they are in that period where tests would not be able to detect the disease if present. Testing all slaughter cattle for BSE could produce an exceedingly high rate of false negative test results and offer misleading assurances of the presence or absence of disease.

> Simply put, the most effective way to detect BSE is not to test all animals, which could lead to false security, but to test those animals most likely to have the disease, which is the basis of USDA's current program.

https://www.usda.gov/topics/animals/bse-surveillance-informa...

s1artibartfast5 tahun yang lalu

Thank you for the context!

rvba5 tahun yang lalu

Sounds like a way to not test anything at all.

I really try to stay away from politics, but sounds as something made by some bribed political appointment.

jfengel5 tahun yang lalu

I'm curious why you'd say that. It sounds to me like a bureaucrat pointing out a scientific fact about the testing.

I'm not an expert on testing for prions, but it doesn't surprise me that the test isn't good enough to pronounce an animal disease-free. And if that's correct, it's very much their purview to make it clear that advertising it as disease-free would be false.

Most of the work at US agencies is done by career employees who are largely insulated from changes at the top. Politics certainly happens, but rarely at the level of individual decisions like this.

Such agencies certainly aren't immune to regulatory capture, and it's conceivable that it's the case here. The scientists and bureaucrats who wrote that have likely worked with the cattle industry their whole careers, and have many personal contacts. Same for their bosses.

But this memo turns on a specific technical point. If the tests aren't good enough, then they're not good enough. It wouldn't take regulatory capture for them to reach that conclusion, and this memo is exactly how they'd say it. It's also what it could sound like if they were being influenced by industry, but I'd say that the burden of proof is on somebody making that claim.

rvba5 tahun yang lalu

"Most new cars sold in USA have breaks that work. It doesnt make sense to test them since this would give the public a wrong impression that cars are safe".

If 3,4 million new cars are sold in USA then probably 99% are generally ok. Because in current world generally most things are ok by default (custom software might be different, but off the shelf software generally works).

But if you would be unlucky 1% where nobody checked the breaks... this would be 34 yhousand cars.

And there is much more cattle raised and consumed so checking everything makes sense.

Not checking anything because 99% will be ok is a pseudoscientific argument.

mannerheim5 tahun yang lalu

> "Most new cars sold in USA have breaks that work. It doesnt make sense to test them since this would give the public a wrong impression that cars are safe".

This isn't what the argument against testing every cow is about. It is really 'You can't test every car's brakes with a test that will say the car's brakes are OK even if those brakes are bad'. If a food product had a toxic ingredient, do you think it's appropriate for a company to run a test that can detect it, say, 50% of the time, and say their product is xyz-toxin-free? What if the test is only effective 10% of the time? 1%?

The USDA didn't decide not to perform no tests for BSE tests at all. They decided to perform tests specifically for those cases where, if the cow had BSE, the test could actually catch it. Again, I don't entirely agree with the USDA's approach, but it isn't unreasonable.

throwaway_isms5 tahun yang lalu

The rational middle ground would be to allow the private testing, but regulate any advertising or marketing of the testing and/or results on the basis it would likely confuse the consumers. If the interest in private testing magically disappears, then the intent is clear and might be a valuable factor for the consumers who might reasonably conclude the private company only had interest in performing testing they knew or should have known was immaterial for the purposes of marketing the testing to consumers as though it were material.

mannerheim5 tahun yang lalu

It's unclear whether or not that's actually prohibited. It looks like Creekstone was the only beef processor who desired to test for BSE, and this was based on them wanting to export their beef to Japan. The rejection was made on this basis.

gojomo5 tahun yang lalu

This is the same error the CDC & FDA made in the early stages of COVID, insisting on fussy rationed tests based on hand-wavy ideas about public misinterpretation. That error likely contributed to hundreds of thousands of preventable deaths by hiding initial community spread for a month or more.

Mass testing, even testing that's mostly at a stage unlikely to detect anything, and only occasionally on less-typical older animals, will provide new useful info. Waiting to test until an a old symptomatic cow "most likely to have the disease" shows up is closing the barn door after the mad cows have already escaped.

The USDA's logic is dangerous like the bureaucratic rationing of COVID tests, early on. At crucial times in early 2020, no matter how suspicious a case/death, and how well it fit COVID symptoms, if the patient hadn't returned from Wuhan in the last few weeks, a test was prohibited. That's a "we don't want to know" policy.

mannerheim5 tahun yang lalu

> Mass testing, even testing that's mostly at a stage unlikely to detect anything, and only occasionally on less-typical older animals, will provide new useful info.

What useful information does it provide? Is there any evidence that testing can detect BSE before symptoms appear? Japan had tested clinically healthy cows until 2017, when they decided to limit testing to symptomatic cows.[0]

> Waiting to test until an a old symptomatic cow "most likely to have the disease" shows up is closing the barn door after the mad cows have already escaped.

Obviously it would be ideal to detect BSE in cows that are younger and not yet symptomatic. But if there's no evidence that this can be done, testing provides a false assurance of safety. Testing older, symptomatic cows will at least give an estimate as to the prevalence of BSE and an indication as to whether safety measures are working.

It's much ado about nothing, anyway, since the US has only ever had four cases of vJCD from BSE, and in each case the individual had spent large amounts of time in countries known for having tainted beef. Perhaps dementia cases are misdiagnosed, but if there were a large danger of BSE/vCJD, one would expect to see those symptoms in a great deal of younger people.

[0]: https://www.fas.usda.gov/data/japan-japan-lift-age-based-bse...

gojomo5 tahun yang lalu

> What useful information does it provide?

The potential for surprise & early detection.

As your quote noted, most cows are slaughtered earlier, so generations could be infected without any detection. If you only test 5yo symptomatic cows, you can never find an outbreak until it's too late, or anything that contradicts the breezy reassurances of the industry-captured USDA.

Again, it's like when the CDC & Fauci were assuring people that there was no significant community COVID spread, when they were also blocking any testing that could detect such spread, by limiting tests only to those that fit their preconceived notion ("only recent returnees from Wuhan").

How much, if any, random surveillance of older asymptomatic cows happens that gives you such confidence in the current testing?

How many meat cows live long enough to get a good read on the symptomatic rate?

Are you sure early mild symptoms of ill health might not just get a cow culled before enough symptoms-to-test arrive, by ranchers who, like the USDA, "would rather not know"?

Your last paragraph seems to reduce to, "we'll know if there's a lot of it, if and when there are a lot of young people with suspicious BSE".

We should aspire to use something other than our young people for early detection. Or at least not block those who want to try it.

mannerheim5 tahun yang lalu

> As your quote noted, most cows are slaughtered earlier, so generations could be infected without any detection.

They couldn't be detected by those tests anyway if they don't have symptoms, or at least not until shortly before symptoms become apparent.

> Again, it's like when the CDC & Fauci were assuring people that there was no significant community COVID spread when they were also blocking any testing that could detect such spread, by limiting tests only to those that fit their preconceived notion ("only recent returnees from Wuhan").

There are a number of differences here. Most importantly COVID tests can detect if you have COVID even when asymptomatic, but BSE tests cannot detect BSE before it is symptomatic.

> How much, if any, random surveillance of older asymptomatic cows happens that gives you such confidence in the current testing? How many meat cows live long enough to get a good read? Are you sure mild symptoms of ill health might not just get them culled before enough symptoms-to-test by ranchers who, like the USDA, "would rather not know"?

> Your last paragraph seems to reduce to, "we'll only know if there's a lot of it if and when there are a lot of young people with BSE".

First, the testing is of symptomatic cows, not asymptomatic cows, and it is deliberately not a random sample, but a sample of the population that is most likely to have it.

Second, my point is that the lack of any cases of young people with vCJD in the US, let alone widespread cases, indicates that it simply isn't the case that we have some phantom population of cows with BSE that are being let through by the USDA.

> Or at least not block those who want to try it.

I agree the USDA shouldn't block it. However, it isn't unreasonable to prohibit marketing based on the use of such tests if they give an unwarranted impression of safety; allowing that would be misleading advertising. When Creekstone applied to do testing, it was precisely for this reason that they applied, and for this reason they were denied.

gojomo5 tahun yang lalu

> They couldn't be detected by those tests anyway if they don't have symptoms, or at least not until shortly before symptoms become apparent.

Your topmost comment mentioned tests can detect BSE "2-3 months before" symptoms. That'd catch more than waiting for symptoms - especially if done broadly! Waiting for symptoms, when the window-of-detection is so small & so late-in-life, & most cows slaughtered young, seems designed for entities that "would rather not know".

> Most importantly COVID tests can detect if you have COVID even when asymptomatic, but BSE tests cannot detect BSE before it is symptomatic.

This contradicts the explicit 2-3 months before symptoms claim you made previously. Also, being less restrictive about testing could create the necessary market for more sensitive tests. If a cow can have it its full life, but it only reaches detectable levels after years, maybe tests can eventually detect the early presence.

> First, the testing is of symptomatic cows, not asymptomatic cows, and it is deliberately not a random sample, but a sample of the population that is most likely to have it.

Exactly! There's zero testing where it wouldn't already be suspected. So no chance to discover a problem early, or challenge the assumptions limiting testing.

> Second, my point is that the lack of any cases of young people with vCJD in the US, let alone widespread cases, indicates that it simply isn't the case that we have some phantom population of cows with BSE that are being let through by the USDA.

Indeed, but the correlate of that is that the regime you're advocating can thus only detect a problem after a bunch of atypical, or weven "widespread", cases are noticed. That's the signal you're waiting for, before testing more.

That approach sucked for COVID, it'll suck for BSE.

caymanjim5 tahun yang lalu

> But the agency ruled that the BSE test was licensed only for "surveillance" of animal health, and rejected Creekstone's request because it implied "a consumer safety aspect" that was "not scientifically warranted."

I read this as "you can test your animals if you want to, but you can't then use the results to make any claim or statement about them". I suspect they're free to test their animals, but not free to stamp "100% tested BSE-free beef!" on their steaks. I suspect this is what's meant by "surveillance". If that's the case, I agree with the gov't; private testing shouldn't be used to make claims. But it could be used for internal product safety, and if anything comes back positive, the USDA could be brought in to verify and take action.

It's not entirely clear to me from the article.

admash5 tahun yang lalu

The “fun” question is whether prions are present in milk and other dairy products. One might argue that they are not, by virtue of them being largely localized to neural tissue.

Of course, this raises the question of how CWD is being transmitted in elk, since elk don’t eat elk.

“Scientists believe CWD proteins (prions) likely spread between animals through body fluids like feces, saliva, blood, or urine, either through direct contact or indirectly through environmental contamination of soil, food or water.”

https://www.cdc.gov/prions/cwd/transmission.html

Milk is clearly a body fluid, though is curiously absent from the above statement.

And as other comments about the difficulty of sterilizing prions make clear, pasteurization will have no effect.

dnautics5 tahun yang lalu

They arent just localized to neural tissue; they are in high concentration in the tongue, which is probably why grazing animals are so susceptible

jtdev5 tahun yang lalu

Prions persist in soil for years; elk eat where other elk have deposited prions.

spoonjim5 tahun yang lalu

Years? Oh jeez. The soil is covered with brain eating murder proteins?

robbiep5 tahun yang lalu

CWD was innoculated in a deer by insuflating prion bound with clay

TheGallopedHigh5 tahun yang lalu

Insuflation: the act of blowing on or breathing on

titanomachy5 tahun yang lalu

In this case likely means inhalation through the nose

Turing_Machine5 tahun yang lalu

Yeah, the sense of smell is a basal one. The nasal cavity has bazillions of nerve endings that are almost directly wired to the brain.

That's why so many drugs are snorted. It works faster than just about anything else other than direct intravenous injection.

robbiep5 tahun yang lalu

That’s not at all why drugs work quickly through the nose - it has absolutely nothing to do with nerve endings, it has everything to do with a large surface area of mucus membranes allowing quick uptake into the venous system.

Y_Y5 tahun yang lalu

It's just a fancy form of the word "snuffle".

creamytaco5 tahun yang lalu

I stopped eating US beef many years ago for this very reason. The Japanese are not stupid and it's quite obvious that something fishy is going on.

Knowing what we know about the practices of most US beef farms and the countless USDA failures in both detection and enforcement of rules/regulations meant to protect the consumer, I think it's the wise path to take for everyone living here.

Google2345 tahun yang lalu

Q. Why can't we test all beef for BSE safety?

A. BSE tests are not conducted on cuts of meat, but involve taking samples from the brain of a dead animal to see if the infectious agent is present. We know that the earliest point at which current tests can accurately detect BSE is 2-to-3 months before the animal begins to show symptoms. The time between initial infection and the appearance of symptoms is about 5 years. Since most cattle that go to slaughter in the United States are both young and clinically normal, testing all slaughter cattle for BSE might offer misleading assurances of safety to the public.

The BSE surveillance program is not for the purposes of determining food safety. Rather, it is an animal health surveillance program. USDA's BSE surveillance program allows USDA to detect the disease if it exists at very low levels in the U.S. cattle population and provides assurances to consumers and our international trading partners that the interlocking system of safeguards in place to prevent BSE are working.

R0b0t15 tahun yang lalu

This is pretty messed up. As long as you meet legal requirements any additional testing should be permissible, caveat emptor.

eganp5 tahun yang lalu

The risk of cow->human BSE transmission is infinitesimal. Species barriers in prions are surprisingly strong and, for practical purposes, nearly impermeable [0]. In fact, the Prion Protein (PrP) in humans causes multiple distinct diseases including CJD, Kuru, and FFI. Infectious prion confirmations are probably highly influenced by glycosylation [1,2]. Even if this testing wasn't god awfully slow, it's unclear one learns anything from it.

[0] https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2736662/ [1] https://www.cureffi.org/2013/05/05/prion-protein-n-linked-gl... [2] https://www.biorxiv.org/content/10.1101/2021.02.14.431014v2

bigcorp-slave5 tahun yang lalu

What you are saying is dangerous. We had a serious set of exactly these transmission events in Britain only a few decades ago. Clearly the risk is not infinitesimal.

eganp5 tahun yang lalu

Dangerous? The hypothesized connection is not remotely clear. In vitro transformation of human PrP into PrP(Sc) by bovine PrP(Sc) is a necessary for the causal model. The fact that a strong species barrier exists, in vitro and in vivo, points towards a spurious association.

It's understandable why one might infer from the correlation that there's a relationship but this is confounded by observation. The baseline expectation for CJD in a population is 1-2 cases/million [0]. vCJD cases in the UK never exceeded 30/yr [1, Fig 2. a]. Yes, vCJD occurs primarily in younger people - but that's circular as vCJD is defined as CJD-like disease with early onset. If cross-species transmission were common, one would expect a similar spike in age >40.

The incidence of vCJD, even at its peak, was exceedingly rare. Many things, such as observation bias, pollution, or rare side-effects from faddish street drugs could be at fault here. It's tempting to jump to conclusions, a la "eating fats makes you fat," but it's totally unwarranted in the face of the in vitro and in vivo data demonstrating strong species barriers.

[0] https://www.cdc.gov/prions/cjd/occurrence-transmission.html [1] https://link.springer.com/article/10.1007/s00401-020-02153-7

bigcorp-slave5 tahun yang lalu

What happened in the 1990s in Britain?

Google2345 tahun yang lalu

Didn’t a huge number of cows get infected back then with only a small number of cases?

s1artibartfast5 tahun yang lalu

>The risk of cow->human BSE transmission is infinitesimal.

I don't think your sources support this conclusion. bovine spongiform encephalopathy (BSE) rarely passes the species barrier, but it is difficult, if not impossible to estimate what the rate is in the absence of mitigations.

Google2345 tahun yang lalu

Don’t we know already? In the UK weren’t millions of cows infected and eaten for years with only a few hundred human transmissions?

s1artibartfast5 tahun yang lalu

There are a plethora of unknowns that make it very difficult to check transmission rate. Transmission is rare, but this could be impacted by lots of factors. There were hundreds of thousands of cows in the UK, but there were also testing and culling programs to try to prevent them from entering the food supply. There were also regulations on which parts of the cows could be used for human consumption.

Last but not least, there are huge uncertainties around the diagnosis rate of mad cow in humans. For example, a 2013 study (1) found that 1 in 2000 people in the UK now have Creutzfeldt-Jakob disease.

Based on this, it could be possible that transmission rates are very high if a human were to eat mad cow nervous tissue.

1) https://www.bmj.com/content/347/bmj.f5675.full

eganp5 tahun yang lalu

This study is looking at the appendix, not any brain tissue. Without considering symptoms, it's possible that altered PrP expression and/or folding in the appendix does not cause vCJD or result from exposure to PrP(Sc).

Also, these antibodies are not very selective for a given confirmation. Idk how this made it into the BMJ because standard in the PrP field is a protease resistance assay given the issues with Ab specificity.

s1artibartfast5 tahun yang lalu

This article is from the UK National Centre for Infectious Disease Surveillance and Control and is cited by by the NHS and widely in the literature.

I am not an expert in the field, but it seems relevant to consider latent or asymptomatic PrP expression when assessing how often it crosses the species barrier.

reaperducer5 tahun yang lalu

Considering the history of the meat industry, I'm OK with not allowing the farms to police themselves.

macksd5 tahun yang lalu

I think there's a pretty fundamental difference between "allowing farms to police themselves" and actually prohibiting them from doing voluntary testing for diseases. I would compare it to the difference between eliminating a need for prescriptions and prohibiting people from getting screened for skin cancer on the regular.

Is there a better reason to prohibit voluntary testing than what is included in the article?

rapind5 tahun yang lalu

Are they being prohibited from testing though, or just prohibited from making safety claims based on those tests?

CryptoPunk5 tahun yang lalu

Prohibited from testing.

shkkmo5 tahun yang lalu

That isn't what is happening. The USDA is prohibiting suplemental testing. The allegation is that this is because that might reveal cases of infection that would show the USDA testing policies are not sufficient.

Thus the USDA is placing their reputation over the health of US citizens.

vkou5 tahun yang lalu

Another poster makes the argument that the USDA does not prohibit supplemental testing, it just prohibits making safety claims on the basis of supplemental testing.

I find their argument far more persuasive than yours. Is there more to yours?

StanislavPetrov5 tahun yang lalu

No, the USDA prohibited testing. The reason they offered for prohibiting the testing was purportedly their concern for false safety claims. They didn't merely prohibit what they consider to be misleading claims, they used their regulatory power to block the testing.

>It asked the USDA for permission to buy test kits from a California company that made the equipment in France. However, under the Virus-Serum-Toxin Act, the government can regulate the manufacturing and sale of “any virus, serum, toxin, or analogous product for use in the treatment of domestic animals.” The USDA deemed the “rapid BSE test” a “treatment” under the Act and banned Creekstone from buying test kits from Bio-Rad.

https://www.courthousenews.com/usda-can-block-testing-for-ma...

shkkmo5 tahun yang lalu

The persuasivity of an argument does not guarantee its truthfulness.

There are plenty of sources, including court documents, that verify that the USDA was trying to prevent private testing, not just claims being made on the basisnof private testing.

The ban on testing was justified by the USDA based on the potential for safety claims, but the ban itself was on testing.

I don't personally buy the argument, if the US testing was sufficient to guarantee safety I see no consumer harm in allowing additional (potentially ineffective) testing that could help a US producer satisfy other countries' import controls.

mannerheim5 tahun yang lalu

Creekstone specifically argued for its use for marketing purposes:[0]

> Creekstone claims to have suffered $200,000 per day in lost revenue as a result of the diminished export market. Stewart Decl. ¶ 17. Moreover, in markets where U.S. beef is available, Creekstone contends that consumer fears about BSE have diminished its sales. See Id. ¶¶ 4, 5 (discussing market surveys in Japan and U.S.). To allay the concerns of consumers and importers, in 2004 Creekstone made a “business decision” to perform the rapid BSE test on each cow it slaughters. ... USDA memorialized its decision to deny Creekstone permission to purchase rapid BSE test kits from Bio-Rad in a June 1, 2004 letter, concluding that “allowing a company to use a BSE test in a private marketing program is inconsistent with USDA's mandate to ensure effective, scientifically sound testing for significant animal diseases and maintain domestic and international confidence in U.S. cattle and beef products.”

Anyway, at the time of the decision, Japan was allowing US exports of beef (this was argued by the USDA in the district court, which they did not pursue on appeal), so their effort was purely a marketing one. For the record, Japan would subsequently go on to ban American beef again, before allowing imports of American beef once more in 2019, after they themselves had scaled back their BSE testing in 2017 to only symptomatic cows.

Would the USDA have allowed Creekstone to use the test if they had said they were going to use it as part of an animal health monitoring measure, which is what the USDA's program is? Maybe, but obviously Creekstone wasn't using it for that purpose if they were wanting to test every cow, including cows for which the test could not possibly detect BSE even if they had it.

[0]: https://www.animallaw.info/case/creekstone-farms-premium-bee...

shkkmo5 tahun yang lalu

You seems to be laboring under several misapprehensions.

> Japan would subsequently go on to ban American beef again, before allowing imports of American beef once more in 2019,

1) The Japanese rules on beef exports did not change significantly between 2006 and 2019. The ban on old beef that was imposed in 2006 was not lifted until 2019. While an argument relating to this was brough up in the original case (that the USDA lost) but not in the appeals case (that the USDA won.)

2) Nothing in the decision in favor of the USDA that I saw was justified on the basis of the purposes of the testing. The decision came down that the judges readings of what authority the USDA has under US law.

So yes, the USDA has the authority to prevent testing and has used it in ways that directly harmed a US business and I don't see how they protected anyone in the process.

Just because our laws give the USDA that authority, doesn't mean that is a good thing.

jtdev5 tahun yang lalu

Considering the history of the USDA, I’m not okay with the USDA having final say in determining what I or my family put on the dinner table.

NationalPark5 tahun yang lalu

https://en.wikipedia.org/wiki/United_States_Department_of_Ag...

This?

jtdev5 tahun yang lalu

No, I'm referring to the regulatory capture and revolving door culture at USDA that has allowed Monsanto et. al., to douse your kids cereal with glyphosate, refuse hemp production in the U.S., water down the Organic Governance Board with corporate shills, etc., etc.

fareesh5 tahun yang lalu

I lived in Fredericton for about 7 years and have been around the province a bit. The air in Moncton is definitely unusual. I wouldn't be surprised if it has something to do with it.

JPLeRouzic5 tahun yang lalu

Could it be the smell of cyanobacteria[1], gathered by tidal bore from the Petitcodiac river[2], specifically by Hall's creek reservoir[3]?

"This reservoir has experienced cyanobacteria algal blooms for many years due to high levels of nutrients combined with a lack of water velocity from damming."

[1] https://toxics.usgs.gov/highlights/algal_toxins/algal_faq.ht...

[2] https://en.wikipedia.org/wiki/Moncton#Tidal_bore

[3] http://www.petitcodiacwatershed.org/?page_id=302&lang=en

fareesh5 tahun yang lalu

Yeah it is definitely something in the water that's awful, the smell is especially pronounced close to it.

w0de05 tahun yang lalu

Ah, it must be malaria!

kgin5 tahun yang lalu

Interesting. In what way is the air unusual?

fareesh5 tahun yang lalu

It is malodorous

ilamont5 tahun yang lalu

One of the questions used to screen blood donors in the U.S. is whether they lived in the U.K. in the early 1990s. If you answer "yes," you can't donate.

The reason is the outbreak there of bovine spongiform encephalopathy (BSE, aka "mad cow disease") and the human variant mentioned in the article, Creutzfeldt–Jakob disease (vCJD) which may lie dormant for decades IIRC.

tibbydudeza5 tahun yang lalu

prions - scary shit.

cpncrunch5 tahun yang lalu

No evidence of prions so far.

Google2345 tahun yang lalu

A similar thing seems to happen in alzeimers (protein conglomerates)

dnautics5 tahun yang lalu

> CJD test came back negative

So a CJD test is usually staining a biopsy with congo red and observing apple-green birefringence in the image. This generally works to detect amyloid plaques, which is common to all prions. So unless there was a lab mistake, the prion is localized elsewhere from where the biopsies are being done (FFI, for example, localizes to a different part of the brain) or they were using some other procedure, it's seems pretty unlikely this is a prion disease.

omgwtfbbq5 tahun yang lalu

Are there other tests to determine prion disease related brain degeneration? Seems hard to believe they can't identify at all what the cause is.

eganp5 tahun yang lalu

Intracranial inoculation of infected tissue is the old-school way to assay for prion activity. Sometimes it takes a few years to get an answer though, especially in cross-species assays [0].

[0] https://link.springer.com/chapter/10.1007/978-1-4614-5338-3_...

jonnycomputer5 tahun yang lalu

So what I've read, suggests that prions do not degrade in the environment very readily. A deer with prion disease can die, decompose, then the prions be absorbed into the vegetation to be re-transmitted to other deer. Naively it would seem that there could be an ongoing accumulation of prions in the environment.

This makes me think of fire policy. Although prions are resistant to temperatures of 200C or so, wildfires often reach much higher temperatures. It would seem, again naively, that a regular regime of fire (e.g. controlled burns) might curtail any such accumulation (as well as have benefits to fire-adapted and fire-dependent plant species). I'm not sure if controlled burns are ever allowed to get that hot though.