The relative consuming disease

The Global Mail has an amazing story about how the last treks to find cases of kuru – a cannabalism-related brain disease – have been completed.

Kuru was passed on by eating the brains of dead relatives – a long finished tradition of the Fore people in Papua New Guinea – and it infected new people through contact with prions.

Prions are misfolded proteins that cause other proteins to take on the infectious misfolding. In the case of kuru it lead to shaking, paralysis, outbursts of laughing and a host of other neurological symptoms as the brain slowly degenerated.

No-one knew prions existed or could exist before kuru. But as the article makes clear, this ‘obscure disease’ of a remote tribe revolutionised our understanding of proteins and how infections could take place.

But the story is how it was discovered is more than just lab tests and the article is a brilliant retelling of the research.

Michael Alpers has been working on the research project since the 60s and recalls some of the episodes:

After each death, he says, “I would go and talk to the family again, and say, ‘Okay?’. They had participated in cutting up bodies in the past — so that was not an unusual activity for them. We had to clear a few people — particularly the women who were wailing. But some of the women stayed. The ones involved put on masks to protect the tissue and I had gloves.

“The father, or a close relative, would hold the head, and I would take the top of the skull off with a bone handsaw. It would take maybe 20 minutes… like cutting an avocado. I would go to particular parts of the brain… take out small cubes. My assistant would hold out the bottle that was relevant, take the lid off, and I’d pop it in.

“Then I’d take the whole brain out and put it in a bucket full of formalin and cotton wool so it wouldn’t be deformed, and put the lid on. All our samples would go into an insulated box. Then I put the skull cap back on, and sewed up. Then we said goodbye… gave everyone a hug, and took off. I did this five times. It was enough.”

It’s a wonderfully written, informative piece. A long and compelling read.
 

Link to article ‘The Last Laughing Death’ (via @mslopatto)

2 Comments

  1. Richard
    Posted November 21, 2012 at 1:20 am | Permalink

    If you are interested in Kuru and its discovery you must read The Collectors of Lost Souls by Warwick Armstrong

    One of the best science books I have read.

  2. Idiot
    Posted January 29, 2013 at 12:54 am | Permalink

    I might add the Wikipedia entry for Alpers’ colleague Daniel Carleton Gajdusek, regarding the statement in the referenced article “Alpers stood by — and remains — fiercely defensive of Gajdusek over a murky episode in later years where he was disgraced and jailed for a year for child molestation. He died in 2008:”

    It reads:

    “In the course of his research trips in the South Pacific, Gajdusek had brought 56 mostly male children back to live with him in the United States, and provided them with the opportunity to receive high school and college education. He was later accused by one of these, now an adult man, of molesting him as a child.

    Gajdusek was charged with child molestation in April 1996, based on incriminating entries in his personal diary and statements from a victim. He pleaded guilty in 1997 and, under a plea bargain, was sentenced to 12 months in jail. After his release in 1998, he was permitted to serve his five-year unsupervised probation in Europe. He never returned to the United States and lived in Amsterdam, Paris, and Tromsø. Gajdusek’s treatment had been denounced from October 1996 as anti-elitist and unduly harsh by controversial Edinburgh University psychologist Chris Brand.[5]

    The documentary The Genius and the Boys by Bosse Lindquist, first shown on BBC Four on June 1, 2009, notes that “seven men testified in confidentiality about Gajdusek having had sex with them when they were boys”, that four said “the sex was untroubling” while for three of them “the sex was a shaming, abusive and a violation”. One of these boys, the son of a friend and now an adult, appears in the film. Furthermore, Gajdusek openly admits to molesting boys and his approval of incest.”


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