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.
Image/CDC
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).
LISTEN: Creutzfeldt-Jakob Disease (CJD) and other prion diseases
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.
Save 87% on Custom Canvas Prints
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
Related:
Do you really want answers to the mystery of TSE? If so visit tseresearchcenter.org and keep an open mind.
The prion is the result of spiroplasma infection and created by the host as protection. TSE type diseases thrive with out prions.
Prions are a red herring and the Enron of science.
“Our studies show a tiny bacterium is involved in the pathogenesis of TSE. Since the discovery of spiroplasma inclusions in CJD brain tissues in 1976, we have confirmed the consistent presence of spiroplasma ribosomal DNA in TSE tissues. DNA sequence data show a link between spiroplasma in CWD and in scrapie. Spiroplasma have been isolated into cell free media from TSE brain and eye specimens confirming the association of this bacterium with TSE. Spiroplasma inoculated into rodents and ruminants produce spongiform encephalopathy and a clinical syndrome remarkably similar to naturally occurring TSE. Recent discovery of biofilm formation by spiroplasma on glass, MICA and stainless steel supports the concept of a bacterial etiology. It is noteworthy that the internal fibril structure of spiroplasma is morphologically identical to SAF.
Spiroplasma entrapped in biofilm are protected from physical and chemical treatments, and not detected by the immune system, therein simulating the biologic properties of the TSE agent. Long conjugate interconnections between organisms in the biofilm suggest a mechanism wherein spiroplasma may form microcolonies in soil. Soil consumption by ruminants is likely responsible for lateral transmission of CWD. Sessile spiroplasma in biofilm produce a functional amyloid that may form a nidus for triggering prion amyloid formation. Continued accumulation of prion amyloid in TSE-affected brains likely occurs by self-assembly. Unfortunately this phenomenon has been interpreted as evidence of protein replication.” Dr. Frank Bastian
Prions are nothing but bits of protein and could never act alone. They could never do the things attributed to them.
Think about it. How do they cross the blood/brain barrier? How do they get from mouth to brain?
Ed Gehrman