THE MYSTERY OF KURU
In 1957, a virologist who had studied several infectious diseases among remote peoples, came to New Guinea to study kuru. Carleton Gajdusek wanted to uncover the cause of this unique and always fatal disease. He searched for sources of toxins in the Fore's diet and environment.
He conducted epidemiological studies and sent samples of brain tissue to the United States to be studied by a neuropathologist. Because there was no sign of inflammation in the bodies or brains of the kuru victims, and because kuru tended to appear within certain families, Gajdusek at first believed kuru was an inherited genetic disorder.
A doctor examines a boy with kuru in New Guinea.
In 1976 Carleton Gajdusek became co-recipient of the Nobel Prize in Medicine for his "discoveries concerning new mechanisms for the origin and dissemination of infectious diseases."
When you look at thin slices of kuru, classic CJD and scrapie brain tissue under the microscope, it is easy to see that they are full of holes. The holes form after misfolded prion proteins kill neurons in the brain. (Photo Credits: clockwise: Kunihiko Kobayashi; University of Iowa; Duke Medical School; University of California, Davis)
In 1959 Gajdusek's work came to the attention of William Hadlow, a research veterinarian who was
studying a remarkably similar disease, called scrapie, in sheep. Like kuru, scrapie was a fatal
disease that gradually destroyed the brains of sheep, leaving the brain full of holes and producing
no immune response. And very importantly, scientists knew that scrapie was infectious.
The similarities between kuru and scrapie led Gajdusek to begin experiments to show that kuru could be transmitted to chimpanzees. He then went on to show that classic Creutzfeldt-Jakob disease (CJD), another spongiform disease in people, was also transmissible.
Ultimately, the rapid spread of kuru was linked to the Fore's funeral rituals: the Fore cooked and ate their dead relatives. This practice was only carried out by the Fore women and children, who lived apart from the men. This explains why men were rarely infected, and why cases appeared within families. The Fore quickly stopped eating their dead, and the spread of the disease stopped. Unfortunately, because of kuru's long incubation time, there are still a few kuru cases among the Fore each year. The people who come down with kuru today are in their 50s and 60s, which means that they have been harboring the disease ever since they ate infected tissue as young children.