MMI 113 Assignment

Kuru

(1)                                                                                                 (2)                                                                                                 (3)
INtroduction

Kuru is a rare prion infection in the brain that has been proven to be fatal. The disease was at it's height in the 1950s to 1960s in the highlands of Papua New Guinea amongst the Fore people. 

Kuru was the first human prion disease discovered and it was reported in 1957 by Dr. Daniel Carlton Gajdusek and Dr. Vincent Zigas.


Dr. Daniel Carlton Gajdusek

September 9, 1923 - December 12, 2011


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Dr. Vincent (Vin) Zigas 

1920 - July 1, 1983



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Key Points
  • Kuru is classified as a Transmissible Spongiform Encephalopathy (TSE) also known as a Prion disease.

  • Kuru is transmitted by the consumption of contaminated brain tissue.

  • Kuru causes the brain tissue to slowly break down and be replaced by cysts.

  • Kuru has three stages: Ambulent, Sedentary, and Terminal.

  • Kuru is fatal as there is no cure.

CLASSIFICATION 

Kuru is classified as a Transmissible Spongiform Encephalopathy (TSE) which is also known as a Prion disease. 

Kuru is caused by the misfolded infectious prion, called PrP.

When it is not misfolded, PrP helps establish normal hemostasis, helps with cell signalling, cell adhesion, neuritogenesis, and protects against cell stress.

Image (6)

Biology and mode of transmission

Similarly to Creutzfeldt-Jakob disease, Kuru is thought to have originally been in a single individual that then went through sudden change creating a pathogenic and infectious agent in their brain.

Kuru is transmitted by cannibalistic practices and can also be transmitted by contact with an open sore or wound. In Papua New Guinea, the brains of deceased members of a family or tribe members were eaten as part of a funeral ritual. Brains that harboured the infectious misfolded proteins caused Kuru. This disease was strictly found amongst the people of the Fore tribe and their neighbouring linguistic groups. It was most prevalent in women and children, as they were often given the brain to eat which is the most infectious.

The disease can also be spread to chimpanzees which allows it to be considered a TSE.

Site of Infection, Pathology, and disease symptoms

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:

  1. Ambulant Stage - This stage comes with action/imitation tremors, ataxic gait, instability, and titubation tremors. 

  2. 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. 

  3. 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. 

A picture of a young boy with kuru                                      the brain when affected

This photo depicts a woman carrying a young girl who has been ailed by Kuru. After contracting the disease the victims would soon no longer be able to walk or stand upright on their own.

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This photo helps to depict the difference between normal brain tissue and that which has been infected by the infectious prion disease, Kuru, which causes cysts to form where normal brain tissue has died. 

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diagnosis and treatment

Kuru is diagnosed by a neurological examination, which includes a medical history, blood testing (to rule anything else out), neurological function testing, and EEGs (which are used to test electrical activity) An MRI scan may be done but it may not be helpful to make a diagnosis.

Kuru can simply be avoided by not participating in cannibalism, specifically in Papua New Guinea, in the 1950s to 1960s as it is widely believed that the last person who had Kuru died sometime between 2005-2009.

As of 2023, there are no treatment options for Kuru or any prion disease. 

Read more about kuru!

Some additional resources to learn more about Kuru!

  1. Kuru: What is it, causes, signs and symptoms, and more. Osmosis. https://www.osmosis.org/answers/kuru

  • This resource is clear and concise and has a good infographic to help with the basics.

  1. Liberski, P. P., Gajos, A., Sikorska, B., & Lindenbaum, S. (2019, March 7). Kuru, the first human prion disease MDPI. https://www.mdpi.com/1999-4915/11/3/232

  • This resource has plenty of photos from the original study on Kuru, and it has more detailed explorations into each one of the sections on this page. 

  1. Klitzman, R. (1998). The Trembling Mountain: A Personal Account of Kuru, Cannibals, and Mad Cow Disease. Plenum Trade.

  • This resource has the first 44 pages available online and it details the experience of Dr. Robert Klitzman and his studies of Kuru and Mad cow disease in person. 

Image Resources

(1) https://storymd.com/journal/xjn6gkvhrw-prion-diseases/page/7qnplcxqdk-kuru-information

(2) https://pdfs.semanticscholar.org/1a75/3f9c9084361374660ce81ce3f9add42c0b13.pdf

(3) https://www.science.org/content/article/small-brains-hint-human-history

(4) and (5) retrieved from: https://www.mdpi.com/1999-4915/11/3/232

(7)  https://www.nobelprize.org/prizes/medicine/1997/7807-physiology-or-medicine-for-1997-animation/

(8) https://learn.genetics.utah.edu/content/basics/prions