The people of the South Fore and those from other groups with whom they intermarried developed kuru, a progressive, invariably fatal encephalopathy with distinct clinical stages. The disease occurred primarily in women and children, because boys customarily did not take part in the rituals beyond the age of 8 years, the authors noted.
Kuru was eventually identified as a prion disease transmitted during the practice of "endocannibalism"--eating the members of one's own family, political, or cultural group.
For the current study, the investigators used kuru as a surrogate for variant CJD, looking at possible incubation periods, pathogenesis, and genetic susceptibility factors among people with kuru in Papua New Guinea.
They began with enhanced surveillance in 1996, scouring the region for all suspected cases of kuru. They took detailed histories of residence and of participation in mortuary feasts, and combined the information with data from serial neurologic exams whenever they were available.