“Was it an invisible miasma that killed these people? Was it an unknown epidemic influence of atmospheric-cosmic-telluric nature, all pervading, inexorable, sneaking into them, poisoning them, killing them?”

These words, penned in the 50s by an Australian doctor, described a mysterious disease that was afflicting a small tribe, the Fore, in New Guinea. The disease, known as Kuru (literally translated as “shaking”), was characterised by uncoordination, trembling, uncontrollable laughter and eventually death.

In the 60s similarities were noted between the deceased from Kuru and sheep who had died from Scrapie. Kuru, it turned out, was an incredibly rare type of disease transmitted through proteins. The infectious proteins, known as prions, had a strange but stable fold in them and had the ability to refold other proteins around it. It affected brains and essentially reformed the neural structure. If you have read Kurt Vonnegut’s Cat’s Cradle, it is like brain ice-nine. Just a protein, not a virus or a bacteria. An entirely normal protein. The Fore tribe were transmitting the disease to each other through their endocannibalistic funeral practices. The disease wouldn’t be passed on via consumption, but if you had any cuts or sores while handling brain, you had a high chance of getting infected. Kuru has a long incubation time, so people were unaware that they were eating infected brain.

The Fore’s practice of cannibalism was heavily discouraged by the colonial government and the disease eventually died out. Although the cannibalism angle to this story is grisly by contemporary standards (few people still practice societally condoned cannibalism), no judgement is implied. What is interesting is how our own proteins reform and kill us: Laughing to death with our own proteins as the joke. Somewhere between the Joker and a zombie film. It becomes very hard not to imagine this disease with a different infection vector.

Another variation of the disease was noted in Italy in the seventies, called Fatal Familial Insomnia, in which the prion seemed to have a genetic origin and caused insomnia until the person literally died of exhaustion:

Mr. MAX: That’s right. I mean I would always be reluctant to rank diseases in terms of horribleness, but I think a case certainly could be made that this disease in many ways – one, because of this insomnia – and anyone who’s ever, you know, suffered insomnia knows just how dreadful a condition it is…

FLATOW: Right, right.

Mr. MAX: …even if the insomnia is simply worries about work tomorrow.

FLATOW: Sure.

Mr. MAX: You stay awake and the clock ticks and the clock ticks. But while this disease has much in common with Alzheimer’s in terms of some of the things going on physiologically in the brain, what makes it quite different from Alzheimer’s is that you – is that many, many of the sufferers of the disease have the ability to understand exactly what’s going on. And even to be in, you know, routine verbal contact with their loved ones at the end, even though they’ve had this extraordinary insomnia. (via)

Studying Kuru and FFI was vital in understanding BSE, more commonly known as Mad Cow Disease, in which a similar prion got passe from infected cow offal to humans, especially in Britain.

Laughing Death | 2009 | Bizarre, True History | Tags: , , | Comments (0)

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