Kuru is a rare neurological disease caused by exposure to prions found in the brain tissue of infected individuals. It was prevalent in New Guinea due to traditional cannibalistic burial practices, causing symptoms such as shaking, bursts of laughter, and catatonia. Kuru provides insight into prions and other transmissible spongiform encephalopathies.
Kuru is a very rare neurological disease that was documented in New Guinea in the 1960s. It is part of a group of conditions called transmissible spongiform encephalopathies (TSEs), which are characterized by damage to the brain that occurs very slowly over time. When someone eventually dies from a TSE, it is discovered that the brain is filled with protein plaques and holes, both of which would have impaired brain function during life.
People get kuru from being exposed to prions, rogue proteins found in the brain tissue of people who have already been infected. Some believe that kuru in New Guinea resulted from traditional burial practices in which people consumed the dead as part of a ritual that was intended to maintain the strength and spirit of the deceased in the tribe. Women and children were commonly offered brain tissue and experienced much higher rates of kuru than men. It is also thought that people may be exposed to prions through open cuts and sores on their hands, which could come into contact with the protein when manipulating brain matter. The rapid decline in kuru rates after the government began recommending alternative burial practices suggests that the theory that this progressive neurological disease was caused by cannibalism is correct, although some scientists continue to dispute this theory.
Once the prion enters the body, it can take years for symptoms of kuru to emerge. In people with this particular TSE, shaking and shaking are experienced; “kuru” actually means “trembling disease”. The patient also experiences bursts of laughter, explaining the alternative name of “laughing sickness”, before sinking into catatonia and becoming unresponsive. Most patients die of pneumonia or pressure sores, the result of prolonged periods in bed. Unlike other TSE patients, kuru patients usually do not develop dementia.
Scientists are interested in kuru and other TSEs because they provide insight into prions and how they are transmitted. Variant Creutzfeld-Jakob disease, another form of TSE seen in humans, has periodically attracted media attention in various regions of the world. This form of TSE appears to be contracted through exposure to the brain tissue of animals, such as squirrels and cattle, and can also be genetic in origin.
The likelihood of contracting kuru is extremely low, requiring intimate exposure to human brain tissue from someone who has an active infection. Other TSEs are also relatively rare in the human population.
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