British beef blues

Curious cause

Down deer, ill elk

Can't happen here?

Laughing death in New Guinea

Identifying disease agents

Menacing microbes

Glossary

The Sad Tale of Kuru

The effects of kuru include congestion of blood vessels, seen here, and in long-standing cases, cortical atrophy, not obvious here. Courtesy: The University of Iowa, Virtual Hospital

PAGE POSTED 23 OCT 1996 In New Guinea in 1950, laughing death was no laughing matter. Desperate to understand the disease, Australian doctor Vincent Zigas penned some prose that was more appropriate to a Gothic novel than a medical treatise. "Was it an invisible miasma that killed these people? Was it an unknown epidemic influence of atmospheric-cosmic-telluric nature, all pervading, inexorable, sneaking into them, poisoning them, killing them?" (see "Laughing death" in the bibliography).

New Guinea, was, and is, a land of 700 tribes, a refuge of stone age cultures in mysterious, cloud-draped mountain rain forest.

"Laughing death," locally called kuru, was a progressive, fatal brain malady that robbed its victims of the ability to walk, talk, and even eat. It had killed dozens of the highland Fore people. At one point, suspecting the cause was a genetic defect, the Australian colonial rulers tried to confine the Fore to their homeland to prevent the genes from spreading.

By 1960, when veterinary pathologist William Hadlow observed similarities between kuru and scrapie, the beleaguered scientists studying the disease suddenly had a frame of reference for the illness.

Kuru infected brain, showing the characteristic holes Courtesy: The University of Iowa, Virtual Hospital

Both diseases caused trembling, uncoordination and certain death. Like scrapie, kuru produced a Swiss-cheesing of the brain.

But it was not the long medical investigation so much as social changes wrought by the introduction of Western technology, religion, and government that halted the epidemic -- by reducing deadly feuds between villages and curbing cannibalism.

Grandma mignon?
Why? Because village rites honored close relatives -- even kuru victims -- by eating them -- after death. This novel understanding of the phrase "family dinner" transmitted the kuru infection either while the bodies were handled or when the relative's remains were eaten.

Mouse brain damaged by scrapie. Such cross-species work allows scientists to test the infectivity of prion diseases. Lajos Laszlo, Eotvos University, Budapest, via Department of Biochemistry, University of Nottingham Medical School.

Curiously, the Fore had been reluctant to eat diseased relatives for fear of laughing death. Like others, they were fooled by the long incubation of kuru, which obscured the link between dinner and disease. But when they began blaming the disease on sorcery, they resumed eating dead relatives. In 1966, Carleton Gajdusek and Michael Alpers at the U.S. National Institutes of Health transmitted kuru to chimpanzees by injecting them with infected brain tissue, proving that the cause was not exclusively genetic and focusing attention on prevention.

As later proved true of AIDS, an incurable disease proved highly preventable.

Prevention worked in New Guinea. The epidemic has tapered off, and no child born since cannibalism ceased has caught kuru. In 1976, Gajdusek shared the Nobel Prize for Physiology or Medicine for the work.

Robert Koch would have been proud. Robert who?