Site of Infection
The site of infection for Kuru is the digestive tract. The infectious prions enter the body through the the consumption of infected tissue or in some cases by contact with it. The prions can infect the entire body, but the highest concentration usually was in the brain tissue, specifically the cerebellum, which helps with balence and coordination.
Pathology/Symptoms
Kuru causes the tissue in the brain to slowly break down and replaces that tissue with clusters of cysts, which look similar to a sponge in appearance.
Since the prion PrP is a naturally occurring protein in our bodies there are no natural defences against it in the case of it misfolding.
Kuru can have a long incubation period which is usually 10-13 years but can take up to 50 years to show symptoms. Once symptoms are present death follows after 6-12 months.
There are three different stages to a Kuru infection:
Ambulant Stage - This stage comes with action/imitation tremors, ataxic gait, instability, and titubation tremors.
Sedentary Stage - This stage starts when the infected can no longer stand on their own and ends when they can no longer sit upright without help, and all previous symptoms worsen. The infected may also show signs of emotional instability, such as depression and uncontrollable laughter.
Terminal Stage - In this stage, all previously mentioned symptoms are worsened, and the infected would also develop dysphagia (difficulty in swallowing), become incontinent, become unresponsive and can no longer speak. At the very end of the disease, the infected also would develop wounds that would no longer heal on their own. Death would follow within 3 months to two years of the first terminal stage indicators.