Medicine: The Laughing Death

In the eastern highlands of New Guinea, sudden bursts of maniacal laughter shrilled through the walls of many a circular, windowless grass hut, echoing through the surrounding jungle. Sometimes, instead of the roaring laughter, there might be a fit of giggling. When a tribesman looked into such a hut, he saw no cause for merriment. The laugher was lying ill, exhausted by his guffaws, his face now an expressionless mask. He had no idea that he had laughed, let alone why. New Guinea’s Fore (pronounced foray) tribe was afflicted by a deadly foe. It was kuru, the laughing death, a creeping horror hitherto unknown to medicine.

Gruesome Ritual. The Fore people, estimated to number 10,000 and only now emerging from the Stone Age, live in a 240-square-mile area 90 miles west of the famed World War II battlefield of Lae (their existence was unknown until 1932). Kuru was first noted in 1951. The disease has not only decimated the Fore, but has become an obsession in their sorcery beliefs. When a kuru victim dies, the kinsfolk pick out a sorcerer suspected of responsibility for the death, do away with him in a gruesome ritual murder called tukavu, in which they pulverize his muscles with stones and bite out his jugular.

Only in the last five years has the Australian administration brought the Fore under regular supervision (it rates them “semi-controlled,” meaning that they usually resist the temptation to plunge a spear into a patrol officer’s back). A year ago the government sent Dr. Vincent Zigas, Estonian-born district medical officer, into the Fore country to investigate kuru. Appalled to find that the disease is invariably fatal, Zigas hurriedly shipped blood and brain specimens from victims to Melbourne’s famed Walter and Eliza Hall Institute, hoping that the laboratories would find a virus cause for the disease. They found none. Next a pathologist, anthropologist, dietitian, psychiatrist and psychologist hit the mountain trails. They eliminated emotional factors as causes of kuru, found no clue to a physical cause.

One Body, One Ax. Last March, a peripatetic U.S. virologist and pediatrician (with a grant from the National Foundation for Infantile Paralysis) appeared in New Guinea. Crew-cut Dr. Carleton Gajdusek, 35, of Yonkers, N.Y., heard about kuru and plunged into its problems. Tramping through rain-soaked forests to Fore hamlets, he rounded up patients for the neat, bamboo-walled native hospital at nearby Okapa Patrol Post. To do autopsies, he had to haggle with victims’ relatives for the bodies. The currency: axes and tobacco. (Dr. Gajdusek got some bodies at the bargain price of only one ax.)

At Okapa, Drs. Gajdusek and Zigas ran the risk of getting kuru themselves (if it should prove infectious); lacking surgical gloves, they did autopsies barehanded. They performed them on a dining-room table in the patrol officer’s quarters, often eating a meal at one end while discussing the kuru-damaged brains lying at the other. They shipped specimens to Melbourne and to the U.S. National Institutes of Health at Bethesda, Md. From 154 patients and their kin, they got a detailed picture of kuru’s course, though no clue to its cause.

No Recoveries. Kuru may strike as early as the age of four. Through childhood it strikes twice as many girls as boys. Among adults the sex ratio rockets to 14 to one: 56% of all patients are grown women. First sign of kuru is a slight trembling of the arms and legs on exertion. At this stage it subsides with rest. But a month to three months later the victim’s head shakes, he begins to sway and stumble, and needs a walking stick for support. Within two months more, he is unable to stand or walk, has to be half-carried to tribal pig feasts. In this stage occur the outbursts which have caused kuru to be dubbed the laughing death. Speech gradually becomes more and more slurred until it is unintelligible. Nearly every victim also becomes conspicuously cross-eyed.

Eventually the kuru sufferer is completely helpless, unable to swallow, capable of only slight movement and feeble grunts. In a native hut, he dies of starvation, infected bedsores or pneumonia. At Okapa’s hospital, Drs. Gajdusek and Zigas have prevented bedsores, and eliminated starvation as a cause of death by intravenous feedings. And still the patients die. No authentic kuru victim has recovered.

In a report for the New England Journal of Medicine Drs. Gajdusek and Zigas list the treatments they have tried: aspirin, sulfas, three antibiotics, cortisone, hydrocortisone, testosterone, phenobarbital, antihistamines, anti-epilepsy drugs, vitamin B, folic acid, liver extract and even a war-gas antidote, British Anti-Lewisite—all to no avail.

Oldsters among the Fore tribesmen remember few cases of kuru before they grew ”grass belong face” (beards). Thus it seems to have become much commoner in the last generation, is estimated to have killed at least 100 Fore in each recent year. It is unknown elsewhere in New Guinea or in the rest of the world. This has led Drs. Gajdusek and Zigas to suspect a genetic defect, with at least a hereditary tendency to the disease. But NIH pathologists at Bethesda have found widespread nerve cell destruction in brains of six kuru victims, suggesting that the cause may be some kind of poisoning. So an intensive, detailed study of everything that the Fore people eat, drink, smoke, or paint on their bodies is under way.

Meanwhile, since white man’s medicine has so far failed them, the Fore see no reason to abandon their own; they still practice tukavu when they think they can get away with it.