Kuru: a Disease Caused by Prions

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Abstract Kuru is a fatal, non-treatable prion disease. Consuming the diseased tissues will result in transfer of the disease. Kuru affects the brain and nervous tissue in humans and lays latent for a long period of time. Growing medical awareness has helped in the decline of the cycle of the disease. History According to Bidon, in 1954, a disease of impending death was reported by W. T. Brown in the Eastern Highlands Province of Papua New Guinea in the Fore tribe. The Fore calls this disease Kuru, which in their language translated to “shiver, shaking, or trembling” (Gadjusek, 1996, pp. 6-33).

It wasn’t until the late 1950s that research started because of the vast rate at which the infection had spread throughout South Fore of the Okapa Subdistrict. At a hospital that was built in 1961 specifically for Kuru patients, observation, and research, brain tissue and blood samples were taken and sent to Melbourne to study (Lindenbaum, 1979. ) Research by Daniel Carleton Gajdusek and Michael Alpers showed that tissue taken from an 11 year old girl and then injected into two chimpanzees can be transferred through infected bio material and it was able to cross a species barrier (Bidon, pp. -2) (Lindenbaum, 1979. ) Causes Kuru is a nervous system disease caused by prions.

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A prion is an abnormally folded protein and when it comes into contact with other proteins causes a chain reaction and spreads the folding to the other proteins. Prions get their name from proteinaceous infectious particle. Theses bundle of proteins are what is found in the brain tissue. The brain tissue dissolves and leaves holes that give it a sponge appearance (Tortora, Funke, & Case, 2010, pp. 392-393,629-631). It is transmitted though eating or contact with open wounds or sores from an infected person.

Ingestion of the prions may be absorbed across the Peyers patch in the gut. Prions do not trigger an immune response and cannot be destroyed by extreme heat or cold (National Institue of Allery and Infectious Disease ). Diagnosis/Prognosis According to Gajdusek, the incubation period is 10 to 13 years, but periods of up to 30 years have been reported. There are three stages of the disease: 1st stage is standing, voice, hands and eyes are unsteady. Also, deterioration of speech begins, tremors, shivering are seen in the patient. Arm and leg pain, headaches are also present.

The second stage symptoms patients cannot walk or support themselves. Severe tremors, no muscle coordination, muscle jerking, depression, outbursts of laughing, and mental awareness is present in the second stage also. The last stage is the terminal stage where death occurs. The patient has slurred speech, incontinence, and there is no brain function. The patient is in an immobile state until death, which is 6-12 months after the first appearance of symptoms (Gadjusek, 1996) (National Instiute for Neurological Disorders and Stoke, 2011) Treatment/ Vaccines

There are no vaccines for Kuru or any of the Prion Diseases. The only treatment that can control Kuru, is discouraging the practice of cannibalism with the Fore tribes. There has been research to understand the diseases immunity. Death is inevitable with this disease. Simon Mead of university College London showed immunity in the inheritance of a genetic prion protein. Research is ongoing (National Instiute for Neurological Disorders and Stoke, 2011) (Gadjusek, 1996). Conclusion

Kuru is a deadly infectious prion disease that affects the human and animal alike. It is contagious and gaining importance in research and knowledge to the scientific community because of the way prion infections are easily transferred and spread. By studying Kuru from the Fore tribal communities of New Guinea and other prion diseases of the world microbiologist and epidemiologist will hopefully learn more about how to develop a treatment or way to start to become skilled and understand how to combat these deadly incurable diseases.

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