Kuru (disease)

In Kuru is a prion disease that occurred epidemic in the 20th century, the people of the Fore in Papua New Guinea and to a lesser extent in some of the neighboring peoples. Furthermore, this also occurred in Tanganyika. The word Kuru comes from the language of the indigenous population and mean muscle tremors.

Signs of disease

The disease manifests itself mainly in movement disorders and typically results within 6 to 12 months after the onset of symptoms to death. In detail, these are among the symptoms by walking and standing uncertainties in terms of cerebellar ataxia, a rhythmic tremor and later in unnatural laughter, which is why the disease is also known as laughing sickness.

Discovery

After the highlands of Papua New Guinea had ever had first contacts with Western civilization until the 1930s, the disease was described in the second half of the 1950s, for the first time and investigated. Particularly worthy made ​​his case DC Gajdusek. The cause of the disease, which was then called by the over 10,000 Fore more than 200 victims annually, one first adopted a genetic cause. Intensive search for environmental toxins or infectious sources were also unsuccessful after the genetic hypothesis had become increasingly unlikely epidemiological reasons. Only after WJ Hadlow the ( Neuropathological ) had recognized the similarity to scrapie already known at that time as transferable scientists studied the transferability of kuru to monkeys under long observation time and was so successful in the 1960s. After further decades of medical, epidemiological and anthropological research is established the hypothesis that kuru was transmitted through Endokannibalismus ( consumption of meat deceased kinsmen ) and the associated therewith handling highly infectious brain at the Fore. Since the cannibalism was banned in 1954 from other, non-medical reasons, including the frequency of diseases declined steadily, to go towards the end of the century to zero.

In retrospect, a beginning of the epidemic at the turn was recognized from the 19th to the 20th century, probably from a single ( sporadic ) event starting. Women and children who are infected when handling the infectious brain parenteral route, ill probably after a short incubation period, while the exclusive peroral intake led to a disease only after decades. Men were probably generally less affected because they took the little infectious muscle meat to him.

Medical importance

The Kuru 's medical history is of great interest, but was noticed by a wider audience, especially after the occurrence of BSE and CJD in the 90s. 2009 doctors discovered that the Fore quickly trained a genetic mutation that prevents the onset of the disease.