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In the early 1950s, when Australian administration patrols first penetrated the mountainous country of the Eastern Highlands of Papua New Guinea, the officers were shown a number of Fore people disabled by a condition of irregular muscular spasms. The Fore called the disorder “kuru”, which in their language meant “trembling with fear” and they held sorcerers responsible for the disease. The Fore practice of seeking out and killing suspected sorcerers meant the disease posed a persistent threat to the establishment of law and order that the administration was attempting to enforce in the region.
The discovery of this disease, common among a remote people recently exposed to European influence, together with the unique ethnic and cultural setting within which it occurred, aroused worldwide media and scientific interest. In 1957, Gajdusek and Zigas detailed the geographic and ethnic background of kuru and the salient clinical features of ataxia and involuntary movement.1 They suggested that the disease predominantly affected the basal ganglia, and stressed its affinities with Parkinson’s disease. Shortly after, following a brief visit, Simpson, Lander, and Robson stressed the significance of the signs of cerebellar disease, but its cause still remained elusive.2
In 1963, I received a letter from Robert Walsh, Dean of the University of New South Wales Medical School, inviting me to investigate a new neurological disease affecting the Fore people of the Eastern Highlands. At the time Walsh was one of six members of the Australian Academy of Science appointed to the Papua and New Guinea Medical Research Advisory Committee to oversee medical research in the Territory. This committee decided to recruit a British trained neurologist as a clinical investigator of kuru. I was very interested in taking up this invitation for two reasons. Firstly, as a passionate amateur entomologist, New Guinea offered a wonderful …