Topic > Kuru, a fatal neurological disorder - 1514

Kuru is a fatal degenerative neurological disorder that appeared in Papua New Guinea in the early 20th century. Kuru belongs to a class of infectious diseases called transmissible spongiform encephalopathies (TSEs), also known as prion diseases. The hallmark of 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 tremble or shiver due to fever and cold, a reference to the tremors of the body which are a classic symptom of the disease and is also known among the Foré as laughter sickness due to the pathological outbursts of laughter which people would show when they were affected by the disease. The discovery of kuru opened new windows in the field of human medicine, was instrumental in the subsequent transmission of other prion diseases, and was one of the major contributions to biomedical sciences in the 20th century. Kuru is caused by prions, an infectious agent composed of proteins in a misfolded form. The disease was the result of the practice of ritual and endocannibalistic funeral practices, in which relatives prepared and consumed the bodies and tissues, including the brain, of deceased family members among the Foré. The brain tissue of individuals affected by kuru was highly contagious, and the disease was transmitted through food or through contact with open wounds or sores. The most striking neuropathological 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 the conversion of conventional proteins into prions. Prions replicate by recruiting normal proteins to their cause, transforming them into a rogue prion-like form that can end up… middle of paper… “Our Town” revolved around the residents of a fictional City of Arkansas showing kuru-like symptoms, which apparently resulted from cannibalistic practices reported from the South Pacific. The episode "Theef" also dealt with a victim apparently suffering from the disease. Since the discovery of the kuru epidemic in New Guinea, a great deal of knowledge about prion diseases has been gained. Scientists admit that there is still a long way to go in this area of ​​research as numerous questions have been answered, but 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 a topic of current study in the hope of defeating these diseases and although Kuru has disappeared, he remains of great relevance to modern neurology.