Fear and Trembling: Prion diseases on Twitter


57 Responses to “Fear and Trembling: Prion diseases on Twitter”

  1. That mousetrap example doesn’t sound like a very good demonstration of misfolding. Of a chain reaction, sure, but the specific form of the sprung mousetrap isn’t what’s causing other mousetraps to turn to the same state.

  2. michael heap says:

    surprised no mention of Amyloid Plaques or post-mortem diagnosis via Congo Red

  3. Stephen Gordon says:

    This was a fascinating article, and I am now going to fully compartmentalize this knowledge away and ignore it so I don’t turn into a shutin.

    Also, needed more weird penises.

  4. Ron Georg says:

    Without an autopsy, it’s very easy to mistake BSE or other prion diseases for Alzheimer’s.  Epidemiological studies show that Alzheimer’s was rare in western cultures until the advent of refrigeration, which is also when meat became the center of our diets, as opposed to a special treat. Studies also show that Alzheimer’s remains relatively rare in cultures with low rates of meat consumption. 

    I know there’s a danger of confusing correlation and causation here, so I hope it’s not specious logic, but it’s entirely possible that consuming animal protein (the source of prions, after all) is the root of our otherwise inexplicably high rates of dementia. 

    Thanks for the story, it’s further confirmation that I’m not just putting on a tinfoil hat by giving up meat.

    • I spoke briefly with Neil Cashman about some of these “meat eating causes Alzheimer’s” theories. He says the evidence supporting that idea is actually pretty poor. I don’t know a lot of detail on it, but, suffice to say, an expert in prion diseases thinks you don’t have as much worry about as you think you do.

      • Ron Georg says:

        Fortunately, I’m not a big worrier, or else that incubation period would weigh heavily–I ate meat for decades. But there are much more scientifically justifiable reasons for giving it up, especially for vascular health. 
        Still, until the experts better understand the mechanisms of various forms of dementia, and can explain the apparent link suggested by the epidemiology, I’m going to hope that my dietary choices may protect my brain as well as my heart.And, off-topic, I recently finished Before the Lights Go Out. While I was aware when I put solar panels on my roof that I was becoming an active participant in the grid, rather than getting off of it, I was fascinated by the complexity of what it means to be part of the grid. I hope that was just the first in a long line of great science books.

      • I always assumed that increased rates of Alzheimer’s were probably because of increased life span due to better medical care. We simply started living long enough that our brains had time to give out. Is there any truth to that, or not?

    • Reg Robson says:

       I’m thinking just correlation not causation. The advent of refrigeration also led to longer lifespans due to generally better kept food. It could simply be that many people didn’t get old enough before refrigeration to get Alzheimer’s in the numbers we’ve seen over the last 70 years or so.

      • Ron Georg says:

        That could account for the historic comparison, but it doesn’t explain the geographic discrepancy. Life expectancy in Japan, a culture fond of rice and veggies, is about five years longer than in the U.S., but Alzheimer’s rates are ten times higher in the States, where there’s a cheeseburger on every corner.

        • TheMadLibrarian says:

           Let’s compare lifespans in both Japan and the US for people on comparable diets.  There are too many variables otherwise.

          • Ron Georg says:

            Absolutely. That’s the problem with epidemiological studies–there are too many variables. It may turn out not to be dietary at all.  Although, when Japanese families move to the United States and adopt a standard American diet, their health patterns quickly start to resemble those of other Americans. 

        • Reg Robson says:

          Sounds like time to set up a study.

  5. Freakpipe says:

    Also, Jay Ingram, while correctly identified as a Canadian journalist, should perhaps be referred to as one of Canada’s pre-eminent science journalists (along with David Suzuki and Bob MacDonald).  

    His work on the weekly science radio show Quirks and Quarks from 1979-1992, followed by his Daily Planet science news show on Discovery from 1995-2011 have educated a generation on the relevance and value of Science!

    Glad to know he’s still rocking it.

  6. Matt Popke says:

    Why did I read any of that, let alone all of it? Something new to fear: twisted molecules. The bit about the incubation period is especially cringeworthy. Every night now, I will lie awake, staring at the ceiling, wondering if there are prions in my brain waiting to put an end to me. I could live to 100 and still lie there, staring at the ceiling, wondering.

    This is worse than stories about parasites. We need a new NSFW-like warning. NSFH: Not Safe For Hypochondriacs. I must go meditate on the possibility of dying from a brain-wasting disease now so that I can go on living my life without fear.

    • xzzy says:

      Your brain began wasting away on its own sometime in your mid 20′s anyways, due to the process of aging.

      Alzheimer’s is effectively senility with the pedal mashed to the floor.

  7. Boundegar says:

    I’ve read the kuru story before, and there was an amusing or horrifying bit of trivia attached.  Apparently, the disease might have been understood sooner, except that Western anthropologists stubbornly held to a doctrine that cannibalism is a myth – despite the knowledge that the Fore term for funeral literally translates, “Cut’em up, cook’em and eat’em.”

    Also, that was one of the most confusing articles I’ve ever tried to read, being in two media at once and tiny grey letters.

    •  Epidemiologicaly speaking the weird thing about Kuru was that it tended to affect more women than men, and it seemed to run in the families that they married into rather than the ones that they were  born into.

      • Anony Mouse says:

        I heard that one reason for this is that their beliefs held that vaginas were dangerous juju, so men consumed genitals during funeral rites to show how tough they were. They reduced their exposure to bad prions via this pattern of consumption, and women bore the brunt of it.

  8. Edwin Lyons says:

    My cousin died for vCJD a few years ago – he went from being an incredibly fit guy in his late 20s to being bed-ridden and seemingly unaware of his surroundings in a matter of months. One of the scariest things I’ve ever seen.

  9. Christina Ward says:

    First:  I ate lunch while reading that.

    Second:  In 2008 two women that I knew (that did not know each other, nor were socially connected in any way) died of Creutzfeld-Jacobs.  Neither had the traditional genetic risk associated with CJ.

    I live in Wisconsin. We have CWD.  I always wondered why this connection isn’t studied more. And then I wonder how many people have died of CJ…I personally knew of those two in a 9 month period. What is that connection between CJ/CWD or is it truly a case of coincidence.

    Third:  For a time, ‘living in the UK’ during the ’90′s was on the list of reasons you could not donate blood; a precaution because of BSE.

  10. JohnQPublic says:

    This is like a Vonnegut book about Ice-9.   How about this:  I read that even if you incinerate the body of an infected cow, the prions causing mad cow will be present in the ashes of the remains and can infect anyone ingesting those prions.

    • jandrese says:

      Seems to me that if there are prions left in the cow you didn’t incinerate it enough.  This is understandable given the difficulty in doing it properly though.  Simply cooking the cow isn’t enough, you need to reduce it to ash, even the bones. 

      Something like a Crematorium could do it if their oven is large enough I think, but a guy out in a field with some logs and a can of gasoline isn’t going to get the job done properly. 

      • JohnQPublic says:

        The text I read involved a crematorium setup, not a guy in a field with a can of gasoline and some sticks:
        from:  http://petergeekie.hubpages.com/hub/BSE-infected-Tallow-and-a-power-station-near-you

        The problem of what to do with potentially BSE infected tallow has been examined by various august bodies, who have rejected land-fill or chemical destruction and have agreed that the only satisfactory and safe method is incineration at high temperature (1000° C). Even at these temperatures the Environment Agency’s own tests confirm that the prion protein responsible for BSE/NvCJD is not necessarily completely destroyed but only the risk of infection is significantly reduced.

        The published results stated “At present, no specific analytical technique exists to test for the presence of the BSE prion in any matter. There is no direct method, therefore, of detecting the prion in either emissions or ash.” It goes on to say “…on the advice of SEAC( Spongiform Encephalopathy Advisory Committee) the agency has made the cautious assumption in its risk calculation that the presence of amino acids, albeit in incomplete sequences, might indicate the presence of some infectivity in the final ash”

        • jandrese says:

          That reads to me like “we don’t have a good test for this stuff, so we are going to err on the side of caution and say it might still be unsafe.”

    • smut clyde says:

       the prions causing mad cow will be present in the ashes of the remains and can infect anyone ingesting those prions.

      Isn’t that how “Return of the Living Dead” ends?

  11. Reed Roberts says:

    Also highly recommended is Deadly Feasts by Richard Rhodes. Really nice look at the science and scientists surrounding the early days of prion (or was that slow virus?) research.

  12. Graham Steel says:

    Good post/Storify.

    Made contact with Jay a while back when I was Information Resource Manager for the CJD International Support Alliance.

    Whilst my slide-deck needs an update it is still relevant:- http://www.slideshare.net/steelgraham/2007-cjdf-presentation-graham11-presentation

    (I lost my brother to vCJD in 1999 so that it how I became involved in the patient support group side of things. I also became very interested in Prion research).

    The second time I went to Washington DC to give a talk at the annual CJD Foundation Conference, prior to leaving the UK, I had already prepared my slides which were science based. I was then asked to give a talk about the patient support group side of things. I decided to combine the two since a lot of work had gone into the science.

    I was quizzed over dinner after giving my talk by the President of the CJD Foundation as to why I didn’t tell her that my talk was not exactly what I was asked to do. I responded with “Did anyone complain?” Since no-one did, she didn’t respond ;-)

    • JohnQPublic says:

       It was a good story but I do think all references to the tweets could be removed from this storify.  The text of the story is a great and interesting account of the disease.  The explanation of how the tweets were sent in the context of a lecture kind of interferes needlessly with the compelling story that it is meant to convey – I’d edit those out to bring the focus closer to the subject matter.

      • oneswellfoop says:

        I agree.  It’s an interesting story, with a side story talking about how you purposefully used a communications medium that is inefficient and ineffective to potentially spread misinformation.  Obvious result is obvious.  Anyone with half a brain could have told you twitter isn’t particularly great for conveying complex and lengthy subject matter.

      • cjporkchop says:

        Disagree. I think it’s useful and interesting to do a ‘post-mortem’ of how a series of communications won/failed in conveying accurate info to the reader.

        • oneswellfoop says:

          Well, my last comment was apparently deleted, so: If you find it useful, good for you.  While the tweets get an A for brevity, they get an F for clarity.  It should have been apparent from the nature, length, and complexity of the material that 148 characters at a time would be insufficient to communicate it.  If you need a post-mortem to tell you that, then….good luck or something.

  13. Jellodyne says:

    Maybe once we grok prion disease more fully the Fore people can return to their more enlightened funerary practices.

  14. Anony Mouse says:

    “But it does seem like prions can get better at infecting a specific species over time. You can infect a mouse with mad cow disease from a cow”

    A minor point, but I don’t think mice can actually contract prion diseases as you present it here, because they don’t have a prion protein at all. Some people also don’t have a prion gene, and because whatever it does isn’t very important (facilitates copper uptake, maybe?) they get on fine and can’t suffer these diseases. I would be very interested to know if I’ve misunderstood this.
    In order to create mouse models for variant CJD and similar diseases, a human prion protein gene has to be inserted into the mouse genome.

    • jandrese says:

      I’ve always thought of prion diseases as a whole class of diseases.  Basically misfolded proteins that can catalyze other proteins to refold in the same shape that cause your body to slowly disintegrate.  The high lethality and poor inter-species communicability (it only works on one specific protein that may or may not exist) has always been the limiting factor in their spread. 

      I wouldn’t be surprised if there are countless numbers of prion type diseases out there that we have never detected because they only affect one person or animal at a time and don’t spread, and also because we don’t have a good way to test for them. 

      Since I read about them, it also suggests an evolutionary reason for the predisposition against cannibalism. 

  15. Jim Jones says:

    I’m actually fascinated by this because of the restrictions that the national blood supply puts on people who were stationed (or family members living with a person stationed) in some European countries during the 1980s during the Mad Cow outbreak. We lived in Germany, one of the countries listed.

    I’ve grown up in a military household that saw giving blood as part of your civic duty. My father has a rare blood type and would receive calls whenever there was a blood drive in his unit or on the post where we were stationed. MANY people, at least at that time, in the military donate blood.

    So, when I graduated high school and moved back to the US, I would give blood  whenever I heard about blood drives in my community. Skip forward a few years, and one of the screening questions asked if potential donors had lived in Europe in the late 1980s. I didn’t think anything of it until, the person running the drive pulled me aside and said that that question disqualified and would continue to disqualify me for the foreseeable future. That was in the mid 1990s and I (nor my father, nor MANY active duty and retired military) have been able to give blood since.

    I’ve brought this up to a number of doctors and some of them weren’t even aware of the restriction.

    Check out the “In-Depth Discussion of Variant Creutzfeld-Jacob Disease and Blood Donation” section of the Red Cross Blood Donation Eligibility requirements page: http://www.redcrossblood.org/donating-blood/eligibility-requirements/eligibility-criteria-alphabetical-listing#arc5 The few that were aware of it thought that is was odd that the ban was still in effect, but, as Maggie pointed out, there is no test for the disease and there as so many unknowns about incubation times that the Red Cross (and other blood banks) can’t take the risk.

    As a result, I’ve followed lots of the general news, watched a few documentaries, and paid attention to popular science coverage of the topic.  When I started hearing reports of CWD, it freaked me out.Anyway, I thought you all might be interested in how the US blood supply, at least, is already addressing prion diseases (or the potential for them) in its policies.

    • Ghostfucker says:

      I lived in Germany as a child in the late 80′s as well, and was always taken out of line during school blood drives when I moved back to the US. Understanding the long incubation period of Mad Cow makes the restriction seem more sensible, when at the time it always seemed like a ridiculous piece of red tape. Of course knowing about the long incubation period isn’t terribly comforting for me!

  16. Øyvind says:

    Articles like this was, and still is, the reason why I read BB every day. Also, only tangentially related; I think kuru was either mentioned or used as basis for an episode of X-files once upon a time.

  17. efergus3 says:

    Lived in Ely, England, ’87 to ’90. So far so good.

  18. The wonderful Canadian show “Regensis” had a plot line surrounding prion disease in Season 1, episode 4.  
    The characters are all employees of “NORBAC”, a fictional entity that investigates all kinds of biological epidemiological threats to North America.  Awesome show.

  19. Evan G. says:

    Thanks for this. If I can add my own thoughts about people live-tweeting conferences…I hate it. It can easily become too much clogging up my feed, so I either mute the conference hashtag or unfollow the tweeter for a period of time. And I do this even when I’m interested in the topic–I’d rather read something longer and thought out about the topic than a bunch of rapid fire nibbles of information with no bigger context mixed in with everything else I’m reading. 

  20. I’m surprised that after 40 odd comments to this piece not one person has brought up William Arens and the considerable controversy surrounding the cannibalism theory of the kuru epidemic, in both the medical and anthropological communities. According to Arens, there were defects in the research methodology of Daniel Gajdusek who won the Nobel Prize for his research into Kuru. For example, correspondence written by Gajdusek indicates that he had concluded that the highlanders of New Guinea were cannibals, before conducting any research or field interviews.  Furthermore, he misrepresented photos of people eating pork as pictures of people eating human flesh. A lot of people would say that Gajdusek’s conclusion has been debunked, but I’d probably just say it’s been harshly questioned. 

    There was a fascinating debate – one of the biggest debates in late-20th century anthropology — over whether south seas cannibalism actually existed, or was merely the product of European racist fantasy, primarily carried on (if I recall from correctly) between Marshall Sahlins on one side, and Arens and Gananath Obeyesekere, on the other.

    It’s worth checking out!

  21. pjcamp says:

    Civilized people don’t eat their parents.

    They eat their god.

    Who, by the way, could do with a little cheese or salsa or something. Would some salt diminish the holiness?

  22. fredfraudster says:

    You know the major players in the prion field have all recently been accused of scientific fraud, right? Read it at http://www.science-fraud.org. All the evidence right there for everyone to see for themselves, copying and pasting of western blot images, re-use of microscopy images, faking of data, the whole lot! Click the prion tag down the bottom right of the page for the articles on Claudio Soto, Stan Prusiner and others. It’s all a sham (not to mention thermodynamically impossible!)

    • Antinous / Moderator says:

      Your proof is a link to an anonymous blog written by you. Forgive me if I don’t take you seriously.

      • michael.kovari says:

        If you look at the evidence on science-fraud. org, you would not write this.Anonymous bloggers have very good reasons to remain anonymous. Anonynity does not equal invalidity

        • Antinous / Moderator says:

          It looks like sniping from the sidelines to me. And when linking to your own blog, you generally identify yourself as the blog author. Failing to do so makes it look like astroturf.

  23. Michele says:

    Interestingly several people in the Southern US also suffer from a similar disease, from eating squirrel brains. 
    We concentrate a bit too much on kuru and its exoticism I think (it fits comfortably with the Western barbarian/headhunter narrative we have about South Sea islands), and not enough with similar disease manifestation in the Western world. As you pointed out, it’s not just about cannibals or industrial farming. 

  24. scientist says:

    Dear Dr. Koerth-Baker,
    It might be work repeating that there is reason, first brought forward by the anonymous author of the blog http://www.science-fraud.org, to believe that one of the tenets of the prion idea,that the altered conformation can propagate in vitro, rests at least in part on manipulated image data.  While I understand that as a new media journalist, one might find this anonymity to be problematic, the blogger self identifies as a working scientist who had reviewed numerous grant applications for public funds.  For what it is worth I find this implicit argument for anonymity to be compelling.  I understand that new-journalistic etiquette here hasn’t gelled yet, but I believe that the work alerting readers to issues with the published work constitutes a public service.  In any case it has already served to cast into doubt one of the pillars of the currently accepted view of prion-caused disease, at least among some members of the scientific community.

  25. I_of_Horus says:

    Here’s a clip of a Kuru sufferer

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