DEMON, GENE OR VIRUS? (Investigator 86, 2002 September)
A FATAL CONDITION Kuru is a rare
fatal
condition occurring
in the New Guinea Highlands and first observed in the early 20th
century. The first symptom is an unsteady gait. Then – during about six
months – it progresses to slurred speech, perpetual trembling, muscle
jerks,
outbursts of laughter, loss of balance and coordination, inability to
stand
or eat, and finally incontinence, difficulty in swallowing,
ulcerations,
coma and death. ![]() An
inhabitant of the
Okapa area — "Kuru
country".
The photo was taken by the editor during his stay there THE
DEMONISM/SORCERY
HYPOTHESIS
ONE hypothesis was that KURU is a symptom of "demon possession" or a manifestation of sorcery. This is what indigenous people believed and some Christian missionaries accepted this. They observed that as Christianity spread and discouraged indigenous rituals, cases of Kuru declined.
Jennifer Cooke in Cannibals, Cows & The CJD Catastrophe (1998) gives a similar account of the demon or "sorcery" hypothesis: Belief in
sorcery was
intrinsic to the
culture of these people of the Highlands… A kuru sorcerer bent on
revenge supposedly took something intimate belonging the intended victim – like excrement, hair, bits of clothing or discarded food. This was added to a magic bundle made from bark, sweet potato leaves, grass, a twig and a sorcery stone tied up with cane and vines. The bundle was named and beaten with a stick then placed in a watery, muddy area while a spell was recited. The idea was that as the bundle deteriorated, so did the health of the victim. (p. 10) SCIENTIFIC
RESEARCH –
GENES AND VIRUSES
The first to study Kuru scientifically was Dr. Vincent Zigas who arrived in New Guinea in 1955 and was alerted to Kuru’s existence by Patrol Officer MacArthur. In 1957 Zigas interested Dr. D C Gajdusek who had come to research child development and disease patterns. In March a Research centre, constructed initially of native materials, was set up west of Okapa. Primitive at first, the research facilities improved during the next 10 years after which the place was closed down. The first researchers into Kuru noticed it seemed to run in families. They therefore postulated a genetic disorder – a mutation passed to offpsring. This explanation lost credibility because Kuru was common yet always fatal. A lethal genetic disorder should decrease in incidence as victims die and the proportion of genes responsible for it decreases in the gene pool. The next hypothesis was a slow-acting virus. In 1963 Dr. Gajdusec inoculated 8 chimps with brain extract of Kuru victims. 1½ to 2½ years later 7 of the chimps had Kuru-like symptoms. (New England J. of Medicine, 1967, pp. 276, 392) This suggested a slow virus. However, a virus could not account for the sex-ratio of victims. In 1957 Kuru claimed over 200 lives of whom 30% were children under 15. The sex ratio of child victims was 1:1. Among adults, however, females outnumbered males 25 to 1. What almost all victims had in common was having engaged in the ritual eating of the dead including eating previous victims of Kuru. Eating of the dead was done mainly by women and children. Therefore, if cannibalism transmitted Kuru, it seemed to explain why so few adult males had the disease. Cannibalism was suppressed in New Guinea in the 1950s but continued in remote areas until the late 1960s. If the cannibalism hypothesis is correct then Kuru should decline from the 1960s onward – which it has done. Regarding the hypothesis that cannibalism, including eating of brains of previous Kuru victims, accounts for transmission of Kuru Zigas concluded: "this hypothesis...should be advanced with much hesitation." (The Medical Journal of Australia, 1975, Sep. 20, p.485) His reasons
were:
1. He doubted
that
brains were often eaten
along with the flesh.
2. He had handled internal organs of victims, at times with scratched hands, without catching the disease. Subsequent research suggested that Kuru along with certain other degenerative diseases are both "infectious and inherited". (New Scientist, 1989, April 8 p. 28; Nature, Vol 338, p. 342) THE PROBABLE
ANSWER
Kuru is now considered a member of a class of diseases referred to as "transmissible spongiform encephalopathies" (TSEs) known also as prion disease. Other TSEs include Creutzfeldt-Jacob disease in humans, bovine spongiform encephalopathy (mad-cow’s disease) in cattle, scrapie in sheep and goats, and chronic wasting disease in deer. Prion disease was identified by an American, Stanley B Prusiner, in 1982. Prions are infectious particles, composed of protein, which build up in the cells of the central nervous system and slowly kill them. This was a new theory in biology. Unlike viruses and bacteria prions lacked genetic material (DNA) yet could replicate without it. They replicated by converting normal protein they came into contact with into a different configuration. The process is a chain reaction that progressively damages the brain. If Kuru is a
prion disease
the symptoms are
due to chronic brain and central nervous system degeneration. And the
reason
for the puzzling age and sex distribution of victims is:
The practice of endocannibalism meant only relatives were consumed, which explained why the disease appeared familial in its distribution. (Cooke, p. 17) TSEs, including Kuru, have a long incubation period – symptoms may take several decades to appear. Kuru effects the base of the brain responsible for coordination which accounts for the initial symptoms of unsteady gait, slurred speech and tremors. Recent research
suggests
that prions sometimes
also result from mutations in the human PrP gene that codes for PrPC
protein.
Cells then produce an altered version of this protein. This means that
DNA mutation accounts for some cases of "prion disease" possibly also
Kuru.
The "gene hypothesis" is therefore partly back.
CONCLUSION For decades the cause of Kuru was unknown and debate is even now not finalised. This scientific doubt led some Westerners to accept the interpretation of sorcery. The observation that as Christianity spread Kuru declined is explained by the ban on cannibalism at the same time. Presumably missionaries also discouraged converts from eating dead relatives. The negative correlation – Christian increase/decline in Kuru – therefore seems accidental, not causative. The recovery of
a Kuru
victim (see Mike F.
letter above) when Kuru is always fatal can be explained by
misdiagnosis.
The initial signs of unsteady gait and inability to stand on one leg
can
have many causes besides Kuru!
In conclusion,
whenever
something can't be
explained we should not immediately implicate the supernatural. To do
so
may be premature.
REFERENCES: Cooke, J. (1998)
Cannibis,
Cows & The CJD Catastrophe. Random House. Australia.
(BS)
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