Doctors investigate mystery brain disease in Canada

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

by aluket • 5 years ago

315 288 5 years ago

bbc.com

phendrenad2• 5 years ago

Related to Havana Syndrome?

r721• 5 years ago

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."

londons_explore2• 5 years ago

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

andai• 5 years ago

The long tail...

londons_explore2• 5 years ago

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?

Brakenshire• 5 years ago

“ 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-...

jokoon2• 5 years ago

I was scared for a bit.

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

drran• 5 years ago

How and where you were able to read this?

jokoon2• 5 years ago

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.

TorKlingberg• 5 years ago

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_account2• 5 years ago

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.”

callamdelaney2• 5 years ago

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

hobbescotch• 5 years ago

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

cpncrunch2• 5 years ago

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

rvz• 5 years ago

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).

forgotmypw173• 5 years ago

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

wobblyasp2• 5 years ago

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.

throwaway3169436• 5 years ago

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

giantg28• 5 years ago

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).

withinboredom2• 5 years ago

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...

sirsinsalot2• 5 years ago

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.

withinboredom2• 5 years ago

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.

Cthulhu_• 5 years ago

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.

Sunspark• 5 years ago

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.

peteretep3• 5 years ago

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

medstrom4• 5 years ago

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

regularfry6• 5 years ago

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.

sirsinsalot2• 5 years ago

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?

StavrosK2• 5 years ago

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

Hypergraphe2• 5 years ago

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.

rndude• 5 years ago

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.`

neartheplain• 5 years ago

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

basicplus2• 5 years ago

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

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

petre2• 5 years ago

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.

colechristensen• 5 years ago

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.

LorenPechtel2• 5 years ago

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

Hypergraphe2• 5 years ago

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

stormdennis2• 5 years ago

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.

stuff4ben2• 5 years ago

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.

gadders• 5 years ago

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.

eloff2• 5 years ago

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.

tauntz• 5 years ago

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

medstrom4• 5 years ago

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

I really need to grow my own food.

Xcelerate2• 5 years ago

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.

senthil_rajasek• 5 years ago

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/

TearsInTheRain• 5 years ago

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?

codingbeer• 5 years ago

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.

medstrom4• 5 years ago

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.

piker• 5 years ago

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)

giantg28• 5 years ago

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.

dcolkitt• 5 years ago

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.

marcosdumay• 5 years ago

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.

userulluipeste• 5 years ago

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.

jeswin• 5 years ago

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.

jeffkeen• 5 years ago

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

hyko• 5 years ago

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

one2three4• 5 years ago

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?

NoGravitas• 5 years ago

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

thehappypm3• 5 years ago

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

throwaway3169436• 5 years ago

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.

orblivion• 5 years ago

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?

arisAlexis• 5 years ago

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

phobosanomaly• 5 years ago

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

andy_ppp2• 5 years ago

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.

wobblyasp2• 5 years ago

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

devit• 5 years ago

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_ppp2• 5 years ago

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

anonymouse0085• 5 years ago

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.

tsimionescu2• 5 years ago

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?

callamdelaney2• 5 years ago

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.

throwaway3169436• 5 years ago

The age range starts at 18 for this outbreak.

psyc• 5 years ago

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

HDMI_Cable2• 5 years ago

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

tcbawo• 5 years ago

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 years ago

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?

GioM• 5 years ago

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

bluGill2• 5 years ago

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.

rubicon332• 5 years ago

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...

decasteve• 5 years ago

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_romeo• 5 years ago

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...

loonster• 5 years ago

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

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

eganp6• 5 years ago

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/

admash2• 5 years ago

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...

admash2• 5 years ago

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.

jtdev3• 5 years ago

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

dnautics4• 5 years ago

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

reaperducer• 5 years ago

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

jtdev3• 5 years ago

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.

macksd• 5 years ago

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?

shkkmo6• 5 years ago

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.

creamytaco• 5 years ago

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.

Google2344• 5 years ago

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.

R0b0t1• 5 years ago

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

mannerheim6• 5 years ago

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• 5 years ago

Thank you for the context!

rvba2• 5 years ago

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.

throwaway_isms• 5 years ago

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.

gojomo3• 5 years ago

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.

caymanjim• 5 years ago

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.

eganp6• 5 years ago

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

s1artibartfast5• 5 years ago

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.

bigcorp-slave2• 5 years ago

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.

fareesh2• 5 years ago

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.

kgin• 5 years ago

Interesting. In what way is the air unusual?

w0de0• 5 years ago

Ah, it must be malaria!

JPLeRouzic• 5 years ago

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

fareesh2• 5 years ago

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

ilamont• 5 years ago

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.

tibbydudeza• 5 years ago

prions - scary shit.

cpncrunch2• 5 years ago

No evidence of prions so far.

Google2344• 5 years ago

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

dnautics4• 5 years ago

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.

omgwtfbbq2• 5 years ago

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.

eganp6• 5 years ago

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_...

jonnycomputer2• 5 years ago

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.

Doctors investigate mystery brain disease in Canada

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