What is kuru?

Kuru is a very rare neurological disease that was documented in New Guinea until 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 finally dies from a TSE, the brain is found to be full of protein plaques and holes, both of which would have impaired brain function during life.

People get kuru by 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 was the result of traditional burial practices in which people consumed the dead as part of a ritual 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 could be exposed to prions through open cuts and sores on their hands, which could come into contact with the protein by 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 question this theory.

Once the prion enters the body, it can take years for the symptoms of kuru to appear. In people with this particular TSE, tremors and tremors are experienced; "kuru" actually means "shaking disease". The patient also experiences bursts of laughter, explaining the alternative name "laughing sickness", before sinking into catatonia and becoming unresponsive. Most patients die of pneumonia or bed sores as a result of spending prolonged periods in bed. Unlike other TSE patients, kuru patients do not usually develop dementia.

Scientists are interested in kuru and other TSEs because they provide information about prions and their transmission. Variant Creutzfeld-Jakob disease, another form of TSE seen in humans, has regularly 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 may also be genetic in origin.

The chance of getting kuru is extremely low, as it requires 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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