Kuru, A Fatal Neurolgical Disorder

Kuru is a degenerative fatal neurological disorder appeared in Papua New Guinea in the early twentieth century. Kuru belongs to a class of infectious diseases called transmissible spongiform encephalopathies (TSEs), also known as prion diseases. The hallmark of a TSE disease is misshapen protein molecules that clump together and accumulate in brain tissue. The term kuru derives from the Foré word kuria which means to shake or shiver from fever and cold, a reference to the body tremors that are a classic symptom of the disease and is also known among the Foré as the laughing sickness due to the pathologic bursts of laughter people would display when afflicted with the disease. The discovery of kuru opened new windows into the realms of human medicine, was instrumental in the later transmission of other prion diseases, and was one of the greatest contributions to biomedical sciences in the 20th century.

Kuru is caused by prions, an infectious agent composed of protein in a misfolded form. The disease was the result of the practice of ritualistic, endocannibalistic funeral practices, in which relatives prepared and consumed the bodies and tissues, including brain of deceased family members among the Foré. Brain tissue from individuals with kuru was highly infectious, and the disease was transmitted either through eating or by contact with open sores or wounds. The most striking neuropathologic feature of kuru was the presence of numerous amyloid plaques, which are associated with the pathology of over 20 or more human diseases.

For the prion, replication involves converting conventional proteins into prions. Prions replicate by recruiting normal proteins to their cause, flipping them into a rogue prion-like shape that can go on to...

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... "Our Town" revolved around the residents of a fictional Arkansas town who displayed kuru-like symptoms, which apparently resulted from cannibalistic practices brought back from the South Pacific. The episode "Theef" also dealt with a victim seemingly suffering from the disease.

Since the discovery of the kuru epidemic in New Guinea, a vast amount of knowledge has been gained concerning prion diseases. Scientists admit that there is still a lot of ground to cover in this area of research as numerous questions have been answered, yet many puzzles still remain to be solved. Fortunately, kuru has disappeared in New Guinea, but many prion diseases remain that can attack humans and animals. Prion diseases must continue to be of a current study in the hopes of conquering these illnesses and although Kuru has disappeared, it remains of great relevance to modern neurology.