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Kuru |
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What is Kuru?
Kuru is a rare and fatal brain disorder
that occurred at epidemic levels during the 1950s-60s among the
Fore people in the highlands of New Guinea. The disease was the
result of the practice of ritualistic cannibalism among the Fore,
in which relatives prepared and consumed the tissues (including
brain) of deceased family members. Brain tissue from individuals
with kuru was highly infectious, and the disease was transmitted
either through eating or by contact with open sores or wounds.
Government discouragement of the practice of cannibalism led to a
continuing decline in the disease, which has now mostly
disappeared.
Kuru belongs to a class of infectious diseases called
transmissible spongiform encephalopathies (TSEs), also known as
prion diseases. The hallmark of a TSE disease is misshapen protein
molecules that clump together and accumulate in brain tissue.
Scientists believe that misshapen prion proteins have the ability
to change their shape and cause other proteins of the same type to
also change shape. Other TSEs include Creutzfeldt-Jakob disease
and fatal familial insomnia in humans, bovine spongiform
encephalopathy in cattle (also known as mad cow disease), scrapie
in sheep and goats, and chronic wasting disease in deer and elk.
Is there any treatment?
There were no treatments that could control or cure kuru, other
than discouraging the practice of cannibalism. Currently, there
are no cures or treatments for any of the other TSE diseases.
What is the prognosis?
Similar to other the TSEs, kuru had a long incubation period; it
was years or even decades before an infected person showed
symptoms. Because kuru mainly affected the cerebellum, which is
responsible for coordination, the usual first symptoms were an
unsteady gait, tremors, and slurred speech. (Kuru is the Fore word
for shiver.) Unlike most of the other TSEs, dementia was either
minimal or absent. Mood changes were often present. Eventually,
individuals became unable to stand or eat, and they died in a
comatose state from 6 to 12 months after the first appearance of
symptoms.
What research is being done?
The taj funds research to better understand the genetic,
molecular, and cellular mechanisms that underlie the TSE diseases.
Findings from this research will lead to ways to diagnose, treat,
prevent, and ultimately cure these diseases.
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