Hacker News new | past | comments | ask | show | jobs | submit login
Doctors investigate mystery brain disease in Canada (bbc.com)
315 points by aluket on May 5, 2021 | hide | past | favorite | 303 comments



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...


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...


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.


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.


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.


> 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...


> 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.


> 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.


> 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.


Thank you for the context!


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.


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.


"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.


> "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.


> 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.


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.


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


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


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


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


Insuflation: the act of blowing on or breathing on


In this case likely means inhalation through the nose


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.


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.


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


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.


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.


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


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


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.


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


What happened in the 1990s in Britain?


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


>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.


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?


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


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.


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.


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


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?


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


Prohibited from testing.


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.


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?


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...


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.


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...


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.


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.



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.


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...


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/


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

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


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?


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


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.


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


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


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


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


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.


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.


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.


For which generation?


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.


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.


> 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.


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.


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.


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.


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


> 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.


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


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.


For a new generation of 10000 every day.

https://xkcd.com/1053/


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


> 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.


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


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


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


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.


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


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.


That might be. I'm not an expert.

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


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


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.


Foot


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


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


This really puts it into perspective...


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/


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

I really need to grow my own food.


Make sure to keep the deer out!


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?


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.


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).


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.


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.


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.


> 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.


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.


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


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


> 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.


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.)


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.


> 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.


> 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


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


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.


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


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.


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.


>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.


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.


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.


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.


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)


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


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.


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.


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.


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.


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.


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)


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.


> 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.


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.


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


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.


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.


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.


Is there a blood test for that?


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.


Well they got your sample for testing going forward.


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


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.


This feels like being worried about sharks evolving machine guns.


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


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.


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


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.


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?


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?


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


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.


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.


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


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


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


I thought 200C for an hour was enough.


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.`


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.


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.


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


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.


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.


Destroying prions is eco friendly.


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


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.


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


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...


Are prions contagious through contact or only eating?


That's not good.


Oooof...


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


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

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


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.


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.


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.


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.


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


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?


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


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


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


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


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


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


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.


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


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).


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


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


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.


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


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.


The age range starts at 18 for this outbreak.


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


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


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.


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?


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).


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


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


> 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.


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.


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_...


I was scared for a bit.

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


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


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.”


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.


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.


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.


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


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.


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.


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


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.


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.


How and where you were able to read this?


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.


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 :-(


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


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.


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.


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


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


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


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.


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...


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.


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


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


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


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".


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.


btw this is where the "19" comes from.


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.


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.


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


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.


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


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


The long tail...


“ 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-...


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?


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.


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.


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


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.


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...


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


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.


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.


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.


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


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


Ah, it must be malaria!


Interesting. In what way is the air unusual?


It is malodorous


Guidelines | FAQ | Lists | API | Security | Legal | Apply to YC | Contact

Search: