Human Form Of Prion Disease

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Prion disease, also known as a transmissible spongiform encephalopathy (TSE), was first seen in 1902 within a linguistic group in Papa New Guinea, called the Fore. This human form of prion disease is called kuru and around the 1950’s, it proved to be untreatable and always fatal to those infected by it. At its peak, kuru reached epidemic levels as 200 new cases of kuru were reported annually during the mid-1950’s, contributing to greatly to its death toll of 3000 people (Goldfarb, 2002). The Fore people were mostly affected by kuru and comprised 80% of kuru deaths recorded (Goldfarb, 2002). Unlike neighboring tribes, the Fore linguistic group of Papa New Guinea often conducted a cannibalistic feast of deceased loved ones for the purpose of…show more content…
This can be simply attributed to the fact that adult males seldom participated in the cannibalizing of the dead. In addition to this, when adult males did participate in the mortuary feasts, they often consumed the muscle tissues of the deceased and not the brain, which is the most infectious organ when concerning kuru. Due to the loss of susceptible genotypes and the decline of cannibalistic practices by the Fore people, there were less incidences of Kuru and a decline in kuru mortality rate after the 1950’s. However, kuru-related mortalities, although minute, did persist past this period due to the long incubation time of kuru (mean incubation time ≅12 years). In some cases, patients weren’t afflicted with kuru until 50 years after exposure to the infection. The active phase of the kuru disease can be differentiated into three main stages, each with their own set of unique symptoms: ambulant stage, sedentary stage and terminal stage. The entirety of the active phase of kuru occurs within 12 months. Before any of these stages emerge, the patient experiences preliminary symptoms in the prodromal period such as headaches and joint pain that starts at the knees and ankles then proceeds to the elbows and wrists (Liberski et al., 2012). The brief prodromal period precedes the ambulant stage, which is the longest stage (8 months) and ends when the patient can
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