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An Indigenous Term For Neurodegenerative Disease

Kuru is a fatal neurodegenerative disease caused by a pathogenic protein called a prion. The medical term for prion disease is transmissible spongiform encephalopathy (TSE). As the name implies, it is highly transmissible.

Kuru was discovered by D. Carleton Gajdusek, who won a Nobel prize for his work in 1976. It was also the first human prion disease transmitted to chimpanzees and was classified as a TSE. 

The discovery of kuru and its transmissibility opened new paths of human medicine and raised the alarm about the transmissibility of Creutzfeldt–Jakob disease (CJD). Unfortunately, we now know that all forms of prion disease are infectious.

Stanley B. Prusiner blazed a related path and discovered prions – infectious and deadly proteins that caused kuru and a whole class of disease now known as prion disease. Prusiner earned the Nobel Prize in 1997. 

Kuru was found among the Fore people of Papua New Guinea.The word kuru stems from the Fore word meaning “to tremble” or “shivering.”

The disease spread through ritualistic endocannibalism, where community members cooked and ate the bodies of deceased relatives as a sign of respect and grief. Women and children traditionally consumed the brain tissue, which held the highest concentration of infectious prions, making them overwhelmingly more likely to contract the disease than men.

Endocannibalism (the eating of relatives) was a component of South Fore mourning rituals, in contrast to exocannibalism (the eating of enemies). 

When preparing a body for human consumption, maternal kin dismembered the corpse. They removed the hands and feet, then cut open the arms and legs to strip the muscles. Then they severed the head and fractured the skull to remove the brain for consumption. Although prions spread throughout the body via blood and other fluids, it is believed that the brain and other parts of the nervous system are the most infectious parts of the body.

In July 1959, Gajdusek, received a letter from the American veterinary pathologist, William Hadlow, at the Rocky Mountain Laboratory in Hamilton, MT., which explained the similarities between kuru and scrapie, a slow neurodegenerative disease of sheep and goats known to be endemic in the United Kingdom since the 18th century. A global pattern was beginning to emerge.

Kuru in the Fore tribe

Members of the Fore villages explained that the disease was present for a long time, but it became increasingly severe. Elders remembered a time when kuru did not exist in Fore villages.

In 1961, Australian medical researcher Michael Alpers and anthropologist Shirley Lindenbaum conducted extensive field studies among the Fore. Their research suggested the epidemic may have originated at the beginning of the 1900s from a single individual who lived on the edge of Fore territory and who is thought to have spontaneously developed some form of Creutzfeldt–Jakob disease. 

Alpers and Lindenbaum’s research conclusively demonstrated that kuru spread easily and rapidly in the Fore people due to their endocannibalistic funeral practices, in which relatives consumed the bodies of the dead to return the person’s life force to the village.

The outbreak largely ended when funerary cannibalism ended in the 1960s. It is hoped that the disease has been eradicated, but prion disease and prion contamination are persistent, if not surging, around the world. 

Beyond the Fore tribe, prion disease is known as Alzheimer’s disease, Parkinson’s disease, Creutzfeldt-Jakob disease and ALS, just to name a few. The biggest difference between these different forms of prion disease is the region of the brain that is under attack. Ultimately, deadly prions spread throughout the brain and throughout the entire body, which makes any form of cannibalism a risk for prion exposure. People are infectious long before they are symptomatic.

In other mammals, prion disease is known as mad cow disease, scrapie and chronic wasting disease. It has been found in many species, including deer, elk, reindeer, sheep, camels, mink, elephants and more.

Prion disease is not limited to older people. Even babies and small children can carry deadly prions once exposed. All bodily fluids and cell tissue are infected by prions. Anything that an infected person touches is infected forever. Even skin is infectious, so being a cannibal or vampire today isn’t what it used to be.

Prion disease is not limited to humans. Prion contamination has spread the disease far and wide. Livestock, wildlife and sea mammals also are exposed to prion contamination. As prions migrate, mutate and multiply up the food chain, they become even deadlier. Thanks to the dumping of infectious waste, even plants and vegetables can be infected by prions in soil and water.

Kuru primarily attacks the cerebellum (the part of the brain that controls balance and coordination). However, prion disease can strike anywhere in the brain at first. Prion aversion is our only hope and that is much easier said than done.

prevent and treat prion disease

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Avatar Gary Chandler

Author: Gary Chandler

Gary Chandler is a prion expert. Chandler is connecting the dots to the global surge in neurodegenerative disease, including Alzheimer’s disease, Parkinson’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and other forms of prion disease.