In 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 to eventually die a shivering death.
Well the National Institutes of Health’s (NIH) Neurological Institute had an interest in this strange brain disease. According to Robert Desowitz in his book “Federal Bodysnatchers and the New Guinea Virus”, he details how they discovered it was an infectious agent.
A scientist named Carleton Gadjusek sent the brain of a dead woman back to his lab in Bethesda, Maryland. He homogenized some brain tissue and inoculated it into a chimp named Georgette.
Nothing immediate happens and Georgette was nearly forgotten until a few years later it was noticed that Georgette 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 no virus was seen using a electron microscope. The chimp had what we now know to be kuru, a fatal disease belonging to what we now call the transmissible spongiform encephalopathies (TSEs).
Over 1100 people died of kuru for 10 years from 1957 to 1968. The Fore people were involved in ritualistic cannabilism. 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 TSE, same as Creutzfeldt-Jakob disease, mad cow disease in cattle, scrapie in sheep and 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 “recruit” normal proteins and flip 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 2 years to 2 decades. The symptoms are broken down into three stages.
The first stage or ambulant stage includes unsteadiness of stance, voice, gait, eyes, tremor, and slurring of speech.
The second stage or sedentary stage is defined by the person not being able to walk without support, severe tremors, loss of coordination, muscle jerks, outbursts of laughter and mental slowing.
In the last stage, the terminal stage, the person is unable to sit up, tremors, urinary and fecal incontinence, difficulty swallowing and death.
The above 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 eventually always fatal. Kuru has all but disappeared from the Fore tribe since the practice of cannibalism was stopped years ago.
Originally published on examiner.com on October 28, 2009
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