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Kuru: A true story of cannibalism, science & rogue proteins

THE GLOBAL DISPATCH

Child with kuru, 1960sIN THE HIGHLANDS of Papua New Guinea in the 1950s and 60s, it was noticed that people (mostly women) of the Fore tribe were dying of what was originally thought to be a genetic disorder since it happened among family members.

The disease stole away the affected person’s ability to talk, walk and eat and they eventually died a shivering death. Over 1,100 people died of kuru from 1957 to 1968.

The US National Institute of Health’s Neurological Institute had an interest in this strange brain disease and, according to Robert Desowitz in his book Federal Bodysnatchers and the New Guinea Virus, they discovered it was an infectious agent.

Scientist Carleton Gadjusek (see PNG Attitude’s 2009 obituary of the controversial Nobel prize winner) sent the brain of a dead woman back to his lab in Bethesda, Maryland. He homogenised some brain tissue and inoculated it into a chimp named Georgette.

Nothing immediate happened and Georgette was nearly forgotten until a few years later it was noticed that she was huddled in the corner of her cage shivering with a blank stare of her face.

Gadjusek believed at the time it was caused by a “slow virus”, but none could be seen using an electron microscope. The chimp had what we now know to be kuru, a fatal disease.

The Fore people were involved in ritualistic cannibalism. Upon the death of a person in the tribe, the women would prepare and consume the corpse; removing the arms and feet, stripping the muscle from the limbs and remove and eat the brain.

The women were also known to feed portions of human brains to their children. The brain tissue from a corpse with kuru is highly infectious and a certain death sentence.

Kuru is a transmissible spongiform encephalopathy, or TSE, the same as Creutzfeldt-Jakob disease, mad cow disease in cattle, scrapie in sheep and the chronic wasting seen in elk.

TSEs are also known as prion diseases. A prion is transmissible like a microbe but has no nucleic acid, no DNA or RNA. It is a rogue-protein that recruits normal proteins and flips them into a rogue- prion shape that infect other cells.

The prions clump together and accumulate in the brain eventually giving the brain its characteristic “sponge” or Swiss cheese appearance.

Kuru has a long incubation period ranging from two to 20 years. The symptoms break down into three stages.

The first or ambulant stage includes unsteadiness of stance, voice, gait, eyes, tremor and slurring of speech.

The second or sedentary stage is defined by the person not being able to walk without support, severe tremor, loss of coordination, muscle jerks, outbursts of laughter and mental slowing.

In the last terminal stage, the person is unable to sit up, and there are tremors, urinary and fecal incontinence, difficulty in swallowing and, ultimately, death.

These symptoms are consistent with dysfunction of the cerebellum, the part of the brain that controls these functions.

There is no treatment for kuru or any other prion disease and they are always fatal. Kuru has all but disappeared from the Fore tribe since the practice of cannibalism was stopped years ago.

Comments

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David Wall

Any discussion of kuru is not complete without some reference being given to Dr Vincent Zigas, the first medical graduate to describe and alert the world to its existence.

Dr Jan Saave said he should have shared the Nobel Prize with Professor Carleton Gadjusek.

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