Cannibalism: A Perfectly Natural History(69)
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 the 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.
The Time story then went on to describe how the Fore were “only now emerging from the Stone Age” and that they still practiced cannibalism and the ritual murder of kuru sorcerers (“when they think they can get away with it”). Even when dealing with the scientific aspects of the story, the article’s anonymous author took ghoulish satisfaction in reporting Gajdusek performed autopsies without gloves, atop the same dining-room table where meals were eaten. Additionally, the article continued, the researcher, “had to haggle with victims’ relatives for the bodies” of kuru victims and “he got some bodies at the bargain price of only one ax.” For his part, Gajdusek hated the media coverage and he considered the term “laughing death” to be a “ludicrous misnomer.”
The worldwide media coverage did have at least one positive effect, in that it increased the public’s awareness of the deadly problem facing the Fore. Because of this, universities began to funnel funds into kuru research, and this money helped support a new influx of professional researchers into the region.