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posted by martyb on Wednesday September 07 2016, @08:57PM   Printer-friendly

NPR's "The Salt" column carries the grusome (but interesting) story of a medical mystery:

When researchers made their way to the highlands of Papua New Guinea in the 1950s, they found something disturbing. Among a tribe of about 11,000 people called the Fore, up to 200 people a year had been dying of an inexplicable illness. They called the disease kuru, which means "shivering" or "trembling."

Once symptoms set in, it was a swift demise. First, they'd have trouble walking, a sign that they were about to lose control over their limbs. They'd also lose control over their emotions, which is why people called it the "laughing death." Within a year, they couldn't get up off the floor, feed themselves or control their bodily functions.

Shirley Lindenbaum, a medical anthropologist with the City University of New York, who continues to write about the epidemic, knew it couldn't be genetic, because it affected women and children in the same social groups, but not in the same genetic groups. She also knew that it had started in villages in the north around the turn of the century, and then moved south over the decades.
...
Lindenbaum had a hunch about what was going on: In many villages, when a person died, they would be cooked and consumed. It was an act of love and grief. ... Women removed the brain, mixed it with ferns, and cooked it in tubes of bamboo. They fire-roasted and ate everything except the gall bladder. It was primarily adult women who did so, says Lindenbaum, because their bodies were thought to be capable of housing and taming the dangerous spirit that would accompany a dead body.

Finally, after urging from researchers like Lindenbaum, biologists came around to the idea that the strange disease stemmed from eating dead people.

The story goes on to explain that the disease wasn't spread by a virus or a bacterium, fungus, or parasite. It could survive being boiled into soup, and had no DNA. It was a totally new infectious agent.

It was a twisted protein called "prions," or "proteinaceous infectious particles", that could cause normal proteins in nerve cells to twist just like them, and slowly over long periods of time kill areas of nerve cells in the brain.

The story goes on to cover the similarity to Mad Cow Disease, a species jumping disease also caused by prions, and Chronic Wasting Disease that is affecting mule deer, white-tailed deer, elk, and moose in 21 states.

The CDC is working with public health authorities in Wyoming and Colorado to monitor hunters for signs of prion disease.

If the Zika doesn't get us, the Kuru probably will.


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  • (Score: 3, Interesting) by hendrikboom on Wednesday September 07 2016, @10:55PM

    by hendrikboom (1125) Subscriber Badge on Wednesday September 07 2016, @10:55PM (#398903) Homepage Journal

    If the Zika doesn't get us, the Kuru probably will.

    There are strong indications that Parkinson's and Alzheimer's are also prion diseases.

    Parkinson's seems to start in the dopaminergic neurons in the gut, and then spread to the brain.

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  • (Score: 2) by art guerrilla on Thursday September 08 2016, @12:12AM

    by art guerrilla (3082) on Thursday September 08 2016, @12:12AM (#398927)

    so-o-o-o, we *especially* shouldn't eat them ? ? ?

    • (Score: 2) by Bogsnoticus on Thursday September 08 2016, @07:22AM

      by Bogsnoticus (3982) on Thursday September 08 2016, @07:22AM (#399077)

      Yep. Never eat anyone who can't remember where they put the carving knife, or hands are too unsteady to slice portions off themselves to put into the pot.

      --
      Genius by birth. Evil by choice.
  • (Score: 1, Interesting) by Anonymous Coward on Thursday September 08 2016, @10:09AM

    by Anonymous Coward on Thursday September 08 2016, @10:09AM (#399109)

    It would be interesting to know if the blood brain barrier (BBB) can affect the onset of such diseases. Studies have been shown that gut bacteria produce certain chemicals that are responsible for the expression of proteins that reduce BBB permeability. Without these bacteria the blood brain barrier becomes more permeable to certain toxins which is bad. However this effect is reversible, at least in mice, if you introduce the normal bacteria back into the mice their BBB permeability restores back to normal.

    It would also be interesting to know if probiotics can reduce things like Alzheimer's disease.