Kuru (disease) - History

History

Kuru was first noted in the Fore tribe of the Eastern Highlands Province of Papua New Guinea as Australian administrators explored the area in 1953–1959. Kuru (Keru) was reported by W. T. Brown in Kainantu Patrol Report No 8 of 1953/54 (13 January 1954 - 20 February 1954.) .. "The first sign of impending death is a general debility which is followed by general weakness and inability to stand. The victim retires to her house. She is able to take a little nourishment but suffers from violent shivering. The next stage is that the victim lies down in the house and cannot take nourishment and death eventually ensues." The same reports described the cannibalism practised by the Fore people. It was in the late 1950s that the full extent of the disease was realized, after it had reached large infection rates in the South Fore of the Okapa Subdistrict, though the agent was unknown.

Awande Hospital was built in 1961 in the Eastern Highlands to accommodate kuru patients and research. Kuru was first noted in 1952-1953 by anthropologists R. M. and C. H. Berndt among the Fore, Yate, and Usanufa people. Charles O. Pfarr, Lutheran Medical Services was brought to the area by tribal persons and reported the disease to Australian authorities. Dr. Vincent Zigas, District Medical Officer began observation. Blood specimens and brain tissue were sent to Melbourne. In 1957, Dr. Daniel Carleton Gajdusek of the National Institute of Health joined Dr. Zigas at the research center. Sister Eva Hasselbusch of Germany joined the hospital in 1959 to take care of the patients. Sister Maria Horn of Germany was the first trained sister to work with the doctors to study the disease. By 1968 the hospital ceased to function as a Kuru hospital and was closed (1886-1986, The Lutheran Church in Papua New Guinea by Herwig Wagner and Hermann Reiner).

The disease was researched by Daniel Carleton Gajdusek as part of an international collaboration with Australian doctor (now Professor) Michael Alpers. In the mid-1960s Alpers collected post-mortem brain tissue samples from an 11-year-old Fore girl, Kigea, who had died of kuru. He took these samples to Gajdusek in the USA, who then injected two chimpanzees with the infected material. Within two years, one of the chimps, Daisy, had developed kuru, demonstrating that the unknown disease factor was transmitted through infected biomaterial and that it was capable of crossing the species barrier to other primates.

In 1976 Gajdusek, along with Baruch S. Blumberg, was awarded the Nobel Prize in Physiology or Medicine for showing that kuru was transmissible to chimpanzees. This was the first time that this group of encephalopathies had been demonstrated to be infectious and therefore a major step forwards in their investigation. As kuru is the only epidemic of human prion disease in known human history, it has provided important insights into the variant CJD.