by Bill Schutt
— J. H. P. Murray, Lt. Governor/Chief Judicial Officer, British New Guinea, 1912
In the spring of 1985, veterinarians working in the English counties of Sussex and Kent were puzzled when dairy farmers reported that a few of their cows were exhibiting some peculiar symptoms. The normally docile creatures were acting skittish and aggressive. They also exhibited abnormal posture, difficulty standing up and walking, and a general lack of coordination. Most of the cows were put down and sent on to rendering plants—facilities that process dead, often diseased animals into products like grease, tallow, and bone meal. It wasn’t until the following year that England’s Ministry of Agriculture, Fisheries and Food launched an investigation.
According to research biochemist Colm Kelleher, microscope slides were prepared from the brains of stricken cows and they showed the tissue to be riddled with holes, reminiscent of Swiss cheese. In what would become the first of many unfortunate decisions, the veterinary pathologists who examined the slides blamed the holes on faulty slide preparation. But by November of the following year, researchers knew that the abnormal spaces had once been filled with neurons that had shrunken and died. They also thought that amyloid plaques, the sticky concentrations of a normally non-sticky brain protein, might be a contributing factor to the neuron deaths. The holes and plaques were characteristic of a number of neurological diseases, with sheep scrapie and Creutzfeldt-Jakob Disease (CJD) being the best known of these somewhat mysterious maladies. These and other diseases of their ilk were classified as transmissible spongiform encephalopathies (TSEs) because of the spongy appearance of infected brain tissue.45 The British researchers soon named their new disease Bovine Spongiform Encephalopathy (BSE). The press, of course, would need something a bit splashier. They settled on “Mad Cow Disease.”
By 1987, there were over 400 confirmed cases of BSE, which had spread to cattle herds across England, and while scientists looked for puzzle pieces, nervous government officials (who preferred the term “bovine scrapie”) repeatedly reassured the public that it was safe to eat English beef. And why not, they rationalized, hadn’t scrapie been killing sheep for centuries with no harm to the humans who consumed them? Why then should a bovine version of the disease be any different?
Other researchers, though, were not so sure, and a few of them began comparing BSE to a disease that had killed humans—thousands of them. To these professionals, this particular affliction was still known by its indigenous name, kuru (the trembling disease). Like their UK counterparts, the American media corps had previously scrambled to coin their own inappropriate names for kuru. They settled on “the laughing death” and alternately, “laughing sickness,” at a time when being called “politically correct” meant you had voted for the guy who won.
According to the rash of radio, magazine, and newspaper accounts that followed the discovery of kuru by Western researchers in the 1950s, some of the earliest and most unsettling symptoms of the disease were intermittent bouts of uncontrolled laughter, or what researchers referred to as pathological laughter. But if the symptoms of kuru were disturbing to the mid-20th-century public, the way the disease was said to spread was even worse, for according to those working to unlock the lethal mystery, kuru was spread by cannibalism.
In the early 1950s, anthropologists and medical researchers began arriving at one of the wildest and most primitive regions on the planet. New Guinea, the world’s second largest island, rises from the western Pacific like a dinosaur with a mountainous spinal column. Upon their arrival, the scientists saw no roads and nothing much that resembled their concept of a city or even a town. Instead they found themselves crossing parasite-ridden mangrove swamps and rainforests whose primary inhabitants seemed to be biting insects, terrestrial leeches, and venomous snakes. But even after reaching their destination in the foreboding Eastern Highlands, conditions were no less dangerous, for the researchers had come to study New Guinea’s infamous cannibals.
Numbering approximately 36,000 individuals in the mid-20th century, the Fore (pronounced FOR-ay) spoke three distinct dialects and inhabited some 170 villages situated among New Guinea’s lush mountain valleys. Desiring (and having) little or no contact with the outside world, the Fore practiced the same slash- and-burn agriculture that had sustained them for thousands of years. Currently what made them especially interesting to the researchers was not their lack of contact with the modern world, their farming techniques, or even the reports that they practiced ritual cannibalism. It was the fact that something was killing them—horribly and at an alarmingly rapid rate.
A decade earlier, as post-WWII colonialism extended its reach onto the “primitive” Pacific islands, Australian patrol officers in New Guinea began encountering some of the most isolated of the island’s inhabitants.46 Like the missionaries that had arrived, preached, and often disappeared for their troubles, the Australian officials (whom the Fore called kiaps) encouraged the locals to curtail what they considered some unacceptably bad behavior. Sorcery and tribal warfare, the Aussie officials said, were prohibited. The Fore were also requested to please stop eating each other, which they claimed to do as a way of honoring their dead. Grudgingly, the indigenous people soon agreed to the requests, although today many anthropologists believe that, while most of them complied, others simply concealed their long-held rituals whenever the nosy white people came around.
Unfortunately, many of the Fore were experiencing problems far more serious than the arrival of their pushy new friends. In fact the kiaps were reporting that something akin to a plague was taking place, one that primarily took its toll on women and children. In addition to the uncontrollable laughter, victims experienced tremors, muscle jerks, and coordination problems that gradually gave way to an inability to swallow, and finally, complete loss of bodily control. The Fore responded to their stricken relatives with kindness—feeding, moving, and cleaning them when they could no longer care for themselves. Invariably, though, their loved ones died, all of them—of starvation, thirst, or pneumonia, their bodies covered in bedsores. The mystery disease was killing approximately 1 percent of the population each year.
Fore elders told the foreigners that the sickness resulted from a form of sorcery. The kiaps were informed that the process went something like this: Sorcerers would stealthily obtain an item connected to their intended victim, like feces, hair, or discarded food. After wrapping the object in leaves, they would place it in a swampy area where it couldn’t be found. As the sorcery bundle began to decompose, so, too, the Fore said, would the victim. The elders also told the kiaps that the condition could not be cured or even treated, and they tried to explain that preventing this sort of thing was the main reason they sometimes killed each another. The patrol officers took it all down, and although some of them were quite sympathetic, they drew the line at allowing the Fore to resume the killing of suspected sorcerers. These unfortunates were generally men or boys accused without evidence (usually several days after someone in their own village had died of kuru), then hacked, stoned, or bludgeoned to death in a form of ritual murder known as tukabu.
It made sense to the kiaps that the best way to stop the killing was to gain an understanding of the mystery ailment, and a number of hypotheses were developed. Initially, it was suggested that the deaths were stress-related and had resulted from the Fore making contact with the whites. Others, though, feared that whatever was killing the Fore had a more pathological nature.
By the time Ronald and Catherine Berndt arrived in the New Guinea highlands in 1951, they had already spent years in the field studying Australia’s aboriginal communities. The thought was that the Fore would become another notch on Ronald’s impressive anthropological belt and, at first, things looked promising. The indigenous highlanders threw parties for the couple, reportedly believing them to be the spirits of their dead ancestors, returning to the fold to relearn the language they had apparently forgotten. Soon enough, though, the Fore lost interest in rehabilitating their pale relatives—but no
t in the strange goods they had brought along with them. Fascination soon turned to envy, and not long after the Berndts settled in, they wrote that the locals were “difficult people to deal with,” requiring “payments for stories: salt, tobacco, newspapers, wool strands, matches, razors, and so on.” The anthropologists also reported “plenty of cannibalism.”
“Actually these people are ‘bestial’ in many ways,” Ronald Berndt wrote. “Dead human flesh, to these people is food, or potential food.” He also described cannibalism among the Fore as an outlet for sexual violence, and “orgiastic feast” was a phrase he seemed to regard with fondness.
A decade later, the not-yet-controversial anthropologist Bill Arens commented on Ronald Berndt’s influential 1962 book on social interactions among the Fore. According to Arens, Berndt’s tome, Excess and Restraint, displayed “too much of the former and too little of the latter.” Arens was particularly galled by Berndt’s description of a Fore husband copulating with a corpse as the man’s wife simultaneously butchered the body for a meal. As these things go, she accidently cut off her husband’s penis with her knife. “Now you have cut off my penis!” the man cried. “What shall I do?” In response, according to Berndt, the woman “popped it into her mouth, and ate it. . . . ”
Arens was not alone in his criticism of the Berndts, as others concluded that, while the pair had made some important anthropological contributions, there were more than a few problems with their work. Most of these related to the many instances of outrageous behavior Berndt detailed in his book—coupled with the growing suspicion that he had made much of it up.
As I began my own research on cannibalism, I found it odd that the Berndts’ reputation, especially as it related to their claims about extreme behavior by the Fore, seemed to have recovered quite nicely with the passage of time. In fact, I noticed that the Berndts were cited in many of the more recent papers on kuru and cannibalism as having presented solid evidence that the Fore practiced man-eating.
But more on that later.
Back in the New Guinea Highlands of 1952, relations between the Berndts and the Fore failed to improve during the couple’s second field season. Ronald reportedly slept with a pistol under his pillow, at one point firing it to scatter some villagers who had been bothering him. As for kuru, the Berndts had seen the disease among the Fore but apparently never made any meaningful connections concerning its cause. Within two years of their arrival in New Guinea, the pair left the country for good.
Fortunately, after the Berndts’ inauspicious start, the researchers who followed them had far better luck, some of them initiating studies that would become classics in the fields of anthropology and disease hunting—and eventually garnering a pair of Nobel Prizes.
One of the researchers was Daniel Carleton Gajdusek (GUY-doo-shek), a Yonkers, New York, native and 1946 graduate of Harvard Medical School. Gajdusek had no real interest in practicing medicine but instead chased his fascination with viral genetics and the anthropology of what he called “primitive” communities across the world. He studied rabies and plague in the Middle East, hemorrhagic fever in Korea, and encephalitis in the Soviet Union. Arriving in Melbourne in 1955, the brilliant but eccentric researcher frequently “went bush,” studying child development among the aboriginals and collecting blood serum for several Australian research labs.
Gajdusek flew to New Guinea in 1957 and, with nothing but his own meager funds to support this venture, he began working on a new problem. To a colleague in the United States, Gajdusek wrote:
I am in one of the most remote, recently opened regions of New Guinea, in the center of tribal groups of cannibals, only contacted in the last ten years—still spearing each other as of a few days ago, and cooking and feeding the children the body of a kuru case, the disease I am studying—only a few weeks ago.
But Gajdusek had never seen any actual cannibalism and he had very little real knowledge about kuru. Beyond the stress-related hypothesis, there was some conjecture that the deadly condition might be the result of an environmental toxin. Others believed that kuru was a hereditary disorder. Consequently, Gajdusek got busy. He spent months collecting blood, feces, and urine from the locals. He ran tests on those stricken by the disease and, with the aid of translators, he conducted interviews with victims and their family members.
By mid-1957, Gajdusek was working with Vin Zigas, a medical doctor who had already been gathering information, as well as his own blood samples. That November their initial findings were published in the prestigious New England Journal of Medicine. Kuru, the authors claimed, was a degenerative disease of the central nervous system. They described the clinical course of the disease as well as its curious preference for striking three times as many women as men. The skewed sex ratios were difficult to pick up, however, since more men were being killed for having been kuru sorcerers. For the Fore, ritual murder had become the great equalizer. In what would later become an important observation, Gajdusek also noted that kuru occurred equally in children of both sexes.
The researchers sent off blood and tissue samples for analysis but the results showed no evidence of viral infection, nor did they appear to indicate the presence of a toxin (they had suspected that the Fore were possibly being poisoned by heavy metals in their diet). But a number of the tissue specimens did show something remarkable—although it was as much about what the samples lacked as what they exhibited. After examining the brains of eight kuru victims, scientists at the National Institute of Health (NIH) in Bethesda, Maryland, reportedly found no evidence of inflammation or any immune response. That meant the victim’s body had not been fighting off a pathogenic organism like a virus, bacterium, or fungus. In most cases, at the first signs of a viral, bacterial, or fungal intruder, the body initiates a sustained, multi-pronged defense consisting of non-specific responses like swelling and inflammation, and cell-mediated responses like the production of antibodies—proteinaceous particles that bind to foreign proteins (antigens) found on foreign cells and viruses.47
What the investigators did find was that large regions of the cerebellum (which sits like a small head of cauliflower behind the cerebral hemispheres) were full of holes.
Igor Klatzo, an NIH researcher, had seen a disease like this only once before. “The closest condition I can think of,” he said, “is that described by Jakob and Creutzfeldt.”
Another NIH scientist noticed a similarity between kuru and the transmissible spongiform encephalopathy (TSE) known as scrapie, an unusual infectious agent of sheep. Scrapie, which was present in European sheep by the beginning of the 18th century, was named for one of its symptoms, namely the compulsive scraping of the stricken animal’s fleece against objects like fences or rocks. It had been previously been classified as a “slow virus,” an archaic term for a virus with a long incubation period, in which the first symptoms might not appear for months or even years after exposure. Klatzo and William Hadlow, who had made the kuru/scrapie connection, now suspected that the cause of kuru might also be a slow virus.
At this point, Ronald Berndt tried to reassert himself as the world’s leading authority on the New Guinea highlands and its indigenous inhabitants. Miffed that medical researchers were intruding on what he considered his anthropological turf, he refused to be outdone by upstarts like Gajdusek. Berndt wrote his own article on kuru, reemphasizing the importance of sorcery and resurrecting the original stress-related explanation. Fear alone, Berndt claimed, was probably enough to initiate the irreversible symptoms of kuru.
Fortunately, though, in what many might argue was a strong positive step for kuru research, Berndt and his wife refused to share their “data” and “expertise” with those who were actually doing real research on the disease. Gajdusek, for his part, dismissed Berndt’s baseless assertions, believing instead that the high occurrence of kuru among young children argued against a psychological origin for the disease. He was leaning toward the explanation proposed by genetics professor Henry Bennett, who attempted to explain t
he discrepancy between male and female adults dying of kuru.
Bennett proposed that a mutant kuru gene “K” was dominant (K) in females but recessive (k) in males. Accordingly, only males who were KK (and who had inherited a dominant form of the gene from each parent) died of kuru, while males who were either normal (kk) or carriers (Kk) were unaffected by the disease. Alternately, females who were either KK or Kk died of kuru, while only those females who were normal (kk) were unaffected.
In the end, the fact that kuru victims included equal numbers of male and female children, but few adult males, was deeply troubling to Gajdusek, and it raised serious questions about Bennett’s gene-based disease hypothesis, which was soon abandoned.
By this time the researchers had already been dealing with another problem—this one related to the sensationalized slant the press had given the kuru story. Time magazine, for example, opened its November 11, 1957, article “The Laughing Death” with the following:
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. . . . It was kuru, the laughing death, a creeping horror hitherto unknown to medicine.
