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The Last Laughing Death
November 23, 2012 2:33 PM   Subscribe

Fifty-five years after the first reported cases of kuru, an article looks back at the discovery, study, and cultural context of the prion disease.
posted by zamboni (29 comments total) 27 users marked this as a favorite

 
You know, I so wanted to make a prion disease joke recently about these photos of Bill Nye meeting Actual Cannibal Shia LaBeouf, but I couldn't think what I'd actually say.
posted by radwolf76 at 2:38 PM on November 23, 2012


Funniest article ever; i couldn't stop laughing!
posted by Renoroc at 2:38 PM on November 23, 2012 [2 favorites]


Moral of the story: don't eat people not matter HOW much you love them.
posted by jscott at 2:52 PM on November 23, 2012 [1 favorite]


Not to be confused with Koro, described as: "a culture-specific syndrome in which the person has an overpowering belief that his penis (or other genitalia) will retract and disappear. Also known as shrinking penis, the syndrome is listed in the Diagnostic and Statistical Manual of Mental Disorders. The syndrome occurs worldwide with epidemics and mass hysteria of genital-shrinkage or disappearance occurring throughout history in Africa, Asia, and Europe.[1]"

I'm not sure which is worse, brain-eating Prions or shrinking wangs.
posted by thewalrus at 2:52 PM on November 23, 2012 [2 favorites]


Thanks for posting this! Just last night, I was listening again to my audiobook of The Family That Couldn't Sleep by D.T. Max, which also details the discovery of kuru. I've always been vaguely anxious about prions, since I happened to eat beef in the UK in 1996, but now at least I'm pretty sure I'm safe on that account. They're still terrifying, though -- indestructible disease agents. The first time I listened to this audiobook, I was in bed, and I went to sleep with nightmares about slaughterhouse yards.

When I was thinking about kuru last night, though, I kept losing my train of thought about prions due to the intense creepiness of Carleton Gajdusek. He had a great love of children, in the best and in the worst possible ways. What is one to make of such a figure? But that is another thread, and a horrible one, no doubt.
posted by Countess Elena at 3:29 PM on November 23, 2012


I'm not sure which is worse, brain-eating Prions or shrinking wangs.

WANG shrunk to 0 long ago.
posted by rough ashlar at 3:31 PM on November 23, 2012


"Buy Wangs for just $9.99 at eTrade."

Thanks, but Amazon has a fine selection in the "adult" section, even if a good quality one does cost somewhat more than ten bucks.
posted by thewalrus at 3:33 PM on November 23, 2012 [1 favorite]


"Meanwhile the project overseers invited the ranks of their retired and serving kuru field reporters to nominate what they might like to mark the moment. A medal? A citation?

“They wanted boots,” Alpers says, so that is what they got – good, solid walking boots. After all those miles, up and down all those mountains, on the trail of an elusive killer, the kuru trackers proudly lace up their hard-earned trophies and continue on their way."


A pair of boots! Is it too much to ask of the developed world that we provide basic needs like boots (and clothes, food, clean water, education, etc...) for all of humanity who can't afford them? What is wrong with so much of the developed world?

Sorry. I've slapped myself back to reality now that I realize the USA (my country) won't even do that much for many of its own citizens. Great article. Thanks for the post.
posted by InsertNiftyNameHere at 3:39 PM on November 23, 2012 [1 favorite]


Does "the market" want beef certified "prion free"?

Who cares!

To counter the fears of beef importers as well as domestic consumers, Creekstone developed a plan to test for BSE each of the approximately 300,000 cattle it slaughters each year. Declaration of John D. Stewart ¶ 6 (July 13, 2006) (Stewart Decl.).   The United States Department of Agriculture (USDA), however, asserting authority under the Virus-Serum-Toxin Act, 21 U.S.C. §§ 151-59 (VSTA or Act), denied Creekstone's request to purchase or use a BSE test kit.

Yup, the Government took the position a firm could not inform consumers on the matter of prions.

But your WANGs are still 0.
posted by rough ashlar at 3:49 PM on November 23, 2012 [2 favorites]


Kudos to Alpers and company for driving a horrifying pathogen to the brink of eradication. Doing this is perhaps the highest service scientists can perform for humankind.
posted by justsomebodythatyouusedtoknow at 3:51 PM on November 23, 2012 [2 favorites]


Does "the market" want beef certified "prion free"?

It's a bit more complicated than that. Cattle that are of normal market age (which is what the high-end beef from Creekstone is) are not old enough for the test to work, so the test is essentially useless. Creekstone admitted this but argued that it would allow them to sell to countries that had banned US beef. The USDA was worried that this would discourage these countries from eventually repealing their ban.
posted by 445supermag at 4:34 PM on November 23, 2012 [5 favorites]


I did a 4-person play in middle school as part of a traveling troope that performed at elementary schools. The play was essentially a series of trivia set to weird percussion. One of the bits was on kuru. The bit ended with all of us laughing and then falling off our stools.
posted by jeoc at 4:53 PM on November 23, 2012 [1 favorite]


Pulitzer Prize winner and MacArthur Grant recipient Richard Rhodes authored a dramatic account of kuru, CJD, and BSE in the wake of the 90's UK beef debacle, Deadly Feasts, which I thought a great medical detective story.
posted by j_curiouser at 5:47 PM on November 23, 2012


This was a very interesting read. I can't give blood because I lived in the UK for more than six months in the late 80s. I hadn't been aware that I couldn't donate until I randomly read the list of unacceptable donors when I was invited to a blood drive. I started looking into it more and I was close follower of Stanley Prusiner who won a Nobel prize for his prion research (mentioned in the article). Even now, I joke when forgetfulness hits that it's my mad cow disease. It does have a tremendously long incubation period.
posted by shoesietart at 6:40 PM on November 23, 2012


Kuru kitten
posted by maggieb at 8:07 PM on November 23, 2012


Haven't pretty much all cases of CJD been shown to be the result of something other than consuming beef products? How many actual cases of humans contracting it from eating tainted beef have there been?
posted by Justinian at 9:45 PM on November 23, 2012


What is hypothesized to be gotten from "mad cows" is variant CJD, not regular, and according to the WHO that's still the best hypothesis for transmission.
posted by Sidhedevil at 9:53 PM on November 23, 2012


I did some more Googling and didn't see anything that suggested another transmission route for variant CJD. Justinian, do you remember anything about the papers or articles you saw that had a different hypothesis? I'd be interested in finding them if possible.

The most current info I found suggested about 200 documented cases of vCJD worldwide to date, with 5 of those tentatively ascribed to human-to-human blood transfusion rather than direct infection from animal tissue.

Of course, this is a tiny number compared to the cases of classic CJD during the same period (1 per million per year, per the WHO and the US CDC).
posted by Sidhedevil at 10:13 PM on November 23, 2012


Haven't pretty much all cases of CJD been shown to be the result of something other than consuming beef products? How many actual cases of humans contracting it from eating tainted beef have there been?

"The consumption of food of bovine origin contaminated with the agent of Bovine Spongiform Encephalopathy (BSE), a disease of cattle, has been strongly linked to the occurrence of vCJD in humans...

"The most likely cause of vCJD is exposure to the BSE agent through consumption of food from bovine origin most plausibly contaminated by infected bovine brain or other central nervous system tissue.

"Only four cases of vCJD infection have been associated with blood transfusion: three of these cases developed symptoms of vCJD several years after transfusion, and one died from unrelated causes before developing symptoms of vCJD, but was shown to be infected with vCJD."

posted by Blazecock Pileon at 2:01 AM on November 24, 2012 [2 favorites]


That was an interesting article, thanks for posting. After growing up in the UK, I'm left with the realisation that I'm not clear of vCJD for a while yet. What a horrible disease.
posted by arcticseal at 6:37 AM on November 24, 2012


One of my father's coworkers died of CJD. This was someone my dad had worked with for many years; I remember going to parties at his home when I was a little kid. He had traveled to the UK frequently to see family. The disease was terrible; he went blind and degenerated rapidly.
posted by sevenyearlurk at 7:58 AM on November 24, 2012


Haven't pretty much all cases of CJD been shown to be the result of something other than consuming beef products?

You're probably confusing CJD with vCJD. The overall population incidence of sporadic CJD, the most common form of the disease and one not linked to beef consumption, has hardly changed. vCJD cases, however, rose from none to a signficant portion of overall CJD cases, peaking in 2000, and declining since. [pdf] The fact that they were never the most prevalent form of CJD has no real significance to whether they constituted an epidemic.

In the US, which has never had the epidemic, there have been only three cases at all, none of them linked to US beef products. So you could say "something other than consuming beef products" is the cause of virtually all CJD in the US, but that has no relevance to whether letting our guard down would mean a similar vCJD epidemic in the US.
posted by dhartung at 8:15 AM on November 24, 2012 [1 favorite]


Great article. Thanks for sharing.
posted by (Arsenio) Hall and (Warren) Oates at 8:17 AM on November 24, 2012


I think dhartung is correct and I was confusing CJD with vCJD. Curse you, little v.
posted by Justinian at 11:36 AM on November 24, 2012


Is it typical to call it a variant when the primary difference appears to be mode of transmission? Isn't it the same disease?
posted by Justinian at 11:42 AM on November 24, 2012


After a bit of searching, it looks like it's the same misfolded protein in all the different types of CJD. I guess the reason they call it different things depending on where it comes from is because the proper reaction is different. If it's sporadic (sCJD, the spontaneous type) it's totally random, so as long as you're careful with the remains you shouldn't see further problems. If it's familial (fCJD), then you'll want to test the rest of the family to see if they're at risk. If it's variant (vCJD), you need to look at everyone's diet to see where the contamination is.

Same ultimate disease, and knowing the mode of transmission won't make much difference to the unfortunate victim, but it makes a difference to everyone else who wants to control its spread.
posted by echo target at 12:46 PM on November 24, 2012 [4 favorites]


I found this lecture quite interesting: Prof James Ironside - Prions: The serial killers that attack the brain
posted by (Arsenio) Hall and (Warren) Oates at 4:33 PM on November 24, 2012


Mortuary rites of the South Fore and kuru ... a fascinating anthropological account of the rites of "transumption" (eating of the dead to facilitate their passage to the afterlife) that transmitted kuru.
posted by geneva uswazi at 11:34 AM on November 25, 2012 [1 favorite]


So I've started reading The Family That Couldn't Sleep, linked by Countess Elena above. The title refers to fatal familial insomnia, another prion disease. Interestingly, it's a slightly different misfolding of the same prion that causes CJD. There's only one known protein that can act as a prion, but it can act in different ways depending on the details of the misfold.

If you like reading about horrific incurable neurological conditions causing insomnia, hallucinations, dementia, and death, like I apparently do, you'll also want to check out Awakenings by Oliver Sacks, about the aftershocks of encephalitis lethargica, and The Day of Saint Anthony's Fire, about an unusual case of food poisoning in France in 1951. That was a very long sentence.
posted by echo target at 11:53 AM on November 27, 2012 [1 favorite]


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