- AUCKLAND (AFP) - As Europe
finds itself confronting a worsening madcow disease crisis, for two Pacific
communities the return of degenerative brain diseases has reawakened memories
of their own past terrors.
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- Madcow disease, transmitted by meat through an infectious
agent known as a prion, has been linked to the incurable brain-wasting
Creutzfeld Jacob Disease (CJD) in humans.
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- But long before CJD, two other human prion diseases had
devastating impacts in the Pacific.
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- In the 1940s, 50s and 60s, a disease known as kuru tore
through the Fore tribe of Papua New Guineas Eastern Highlands.
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- Kuru, known as the laughing disease because victims died
with a strange smile on their faces, was found among the 35,000 Fore, who
lived largely on sweet potato and pork.
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- The disease's origins were traced to a bout of anthrax
and pneumonia that hit the Fore's extensive pig herds and which prompted
the Fore to engage in extensive cannibalism of their relatives.
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- Over the next two decades, more than 3,000 people died
of kuru. Women, who generally ate the brain tissues of other humans, were
badly affected: four times the number of women than men contracted the
incurable condition.
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- Death took up to a year. The effect was disastrous, wrecking
the traditional marriage system and producing a severe shortage of women.
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- But in 1960 a pathologist observed striking similarities
between kuru and the sheep brain disease scrapie.
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- With this clue, pediatrician and virologist Carleton
Gajdusek of the US National Institutes of Health showed kuru could be transmitted
to chimpanzees by injecting them with infected brain tissue, providing
the solution to its transmission.
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- In 1976 Gajdusek was awarded the Nobel Prize. Her work
led the Fore to give up cannibalism and no child has since been afflicted.
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- Similarly, a degenerative brain disease called Lytico-Bodig
produced in the indigenous Chamorro of the Micronesian island of Guam a
combination of the worst symptoms of Alzheimer's and Parkinson's at rates
100 times higher than the US incidence for neurological conditions.
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- After US forces retook Guam from the Japanese at the
end of World War II, American doctors identified the hitherto unknown disease.
Symptoms included stiffening muscles, a marked stoop, slow speech, an expressionlessness
demeanour, forgetfulness, hand tremors and dementia.
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- Around 25 percent of all Chamorro deaths over 25 years
were as a result of the disease. This time, men were worst effected.
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- The disease, which had been recorded in folklore for
200 years, found its highest concentration at Umatac and among one particular
clan, the Quinata.
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- Toronto born neurologist John Steele has speculated the
disease originated from eating the seeds of the federico palm or cycad,
which formed a substantial part of the Chamorro diet, particularly during
the Japanese occupation.
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- Although the seeds were poisonous, the Chamorro believed
they were safe once repeatedly washed and ground into a flour known as
fadang which was then used to thicken stews or make dumplings and tortillas.
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- While no definite answer to the mystery of the Lytico-Bodig
has emerged, it has not struck anybody born since 1952 and may now disappear
without a cause ever being found.
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- Steele is confident the cause can still be found, however.
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- And New York neurologist Oliver Sacks, famous for his
book Awakenings, told AFP the Chamorro's treatment of sufferers offered
the world a lesson in dealing with those effected by CJD.
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- "Meeting with the people on Guam, and they way the
take care of the people suffering, has been a remarkably humbling experience,"
he said.
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