FRIDAY, June 23 (HealthDay News) -- Kuru, the human prion disease found in Papua New Guinea related to bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease, can have an incubation period of 50 years or longer, according to a report in the June 24 issue of The Lancet.
John Collinge, F.R.S., of University College London in the U.K., and colleagues identified 11 patients who developed kuru between 1996 and 2004, who were living in the South Fore of Papua New Guinea. All were born before the kuru-spreading practice of cannibalism was abolished in the 1950s. The cannibalism was a funeral rite that was a sign of respect and mourning for dead relatives.
The researchers found that the minimum incubation period of kuru ranged from 34 to 41 years, but was more likely 39 to 56 years, and possibly up to seven years longer. A genetic analysis of the prion protein gene from 10 of the individuals indicated that eight were heterozygous at codon 129, a genotype that has been associated with extended incubation periods and resistance to prion disease.
"Incubation periods of infection with human prions can exceed 50 years," the authors conclude. "In human infection with BSE prions, species-barrier effects, which are characteristic of cross-species transmission, would be expected to further increase the mean and range of incubation periods, compared with recycling of prions within species."
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