The Tale of the Dueling Neurosurgeons: The History of the Human Brain as Revealed by True Stories of Trauma, Madness, and Recovery

by Sam Kean

  The delicacy of the situation made the ruling Australian government tremble. Australia had acquired the highlands after World War I, and politicians there viewed New Guinea as their one chance to become a colonial power. As with most colonial overlords, Australia was motivated by a patronizing wish to “civilize” the natives, combined with a strong lust for profit, and by 1957 it had achieved both ends. Fewer and fewer natives wore penis sheaths or pierced their noses with pig tusks. Papuans now built rectangular homes instead of traditional oval ones, and they abandoned their simple, bamboo-pipe-irrigated yam gardens to slave away on coffee plantations or in mines. At the same time murder rates had dropped off steeply and centuries-old diseases like yaws and leprosy had disappeared. But kuru threatened to upset this pax Australiana by panicking the highlanders and discrediting the government. Colonial officials tried to keep it secret, and they despised Gajdusek for spreading word of it. Hell, for all they knew, Gajdusek himself was spreading the disease by tramping from village to village. So colonial officials tried to restrict his movements within the highlands and even petitioned the U.S. State Department to forbid his travel. They meanwhile played dirty and waged a propaganda war, denouncing him as a “scientific pirate” and threatening other scientists for collaborating with him. One rival taunted Gajdusek that “your name is [now] mud.”

  But Australia was about to learn that Carleton Gajdusek did not lose stare-downs. After throwing a tantrum over the interference, he decided to simply outwork his saboteurs. He’d penetrate more deeply into Fore territory and collect more gallons of blood, urine, and saliva than any five Aussies. Sure enough, within five months Gajdusek had identified hundreds of kuru victims, and he even sweet-talked some families—or bribed them, with knives, blankets, salt, soap, and tobacco—into letting him do autopsies on the victims’ brains. Like a pseudo-cannibal himself, Gajdusek performed some of these autopsies on the kitchen table in his hut, plopping the brains onto his dinner plates and slicing them up like thick white focaccia with a gray-matter crust. He sent most of this precious tissue back to his lab at the National Institutes of Health, in Maryland, but shrewdly also sent samples to Australian scientists, to placate them and undermine the politicians whispering poison into their ears. Eventually Australia realized it would just have to tolerate Gajdusek.

  In the meantime Gajdusek faced another, unexpected obstacle to his work—sorcery. Almost to the last man and woman, the Fore believed that sorcerers caused kuru, and they listened to Gajdusek’s lectures on microbes and genetics with amuse- or bemusement. According to tradition, sorcerers worked their necromancy on personal items, including body discards such as hair, fingernails, and feces. Sorcerers first bound these items with leaves, then cast their spells and buried the bundles in swamps; as the items decayed, so too would the victims’ health. (To be sure, the Fore considered most spells cast in this manner perfectly acceptable, but “making kuru” went beyond the bounds of decency.) To head off sorcerers, the Fore held bonfires to burn their refuse, and also built some of the deepest latrines on the planet. (After doing their business in the woods, they might even carry the turds back to the latrine, for safety’s sake.) And people who had already contracted kuru would hire showy countersorcerers, who chanted and dispensed herbs and forbade patients from drinking water, eating salt, or consorting with the opposite sex. Not surprisingly, people who believed so deeply in sorcery weren’t thrilled about giving up bodily fluids to a stranger. So to convince people of their security, Gajdusek acquired a reassuringly large lock, which he slapped onto his box of samples.

  After Gajdusek collected the samples, they had a dicey future. If he had access to a jeep, he drove them to the nearest patrol station. Often as not, though, an axle had broken or the road had washed out, and he had to dispatch a dokta boi on a multihour hike. It was then even odds whether the freezer at the station was working. Within a few days, hopefully, the blood or brains got loaded onto a plane en route to a city with an international airport. There, a technician could finally pack the samples in dry ice and dispatch them to Maryland or Melbourne or the dozen other places where labs—goaded by Gajdusek—had taken up kuru.

  Neurologists also started trickling into the highlands to examine kuru victims directly and look for signs of brain damage. Some of the tests they administered looked like DUI checkpoint trials, with Fore people walking heel to toe, touching fingers to noses, or standing on one leg and raising both arms. Kuru victims generally failed such tests. Neurologists also tested for certain reflexes. If you tap the skin around an infant’s mouth, she’ll automatically purse her lips; this “snout reflex” makes it easier to suckle a nipple. Similarly, brushing an infant’s palm in certain places will make her fingers curl, a reaction called the hand-grasp reflex. These reflexes disappear during our second or third year as the brain matures and certain other circuits inhibit them. But they can reemerge after brain damage—and often did in kuru victims.

  Based on the battery of tests, neurologists traced much of the initial damage in kuru to the brain’s movement centers, especially the cerebellum. As we’ve seen, a few different patches of gray matter in the brain (e.g., the motor cortex) work together to initiate movement. In addition, the brain’s motor system has some crucial feedback loops to ensure that movements are carried out properly. One key structure in this feedback circuit is the cerebellum.

  As part of the so-called reptile brain, the cerebellum sits way back near the spinal cord, and its wrinkly appearance makes it look like a mini-brain all by itself.

  * It plays an especially important role in coordinating movement and providing balance. In short, the cerebellum collects inputs from all over the brain, including all four lobes. This allows it to monitor the body’s position in space in multiple ways (through touch, vision, balance, and so on). It then checks to see whether the movement you’re executing is anything close to what you intended. If not, the cerebellum pings another brain structure (the thalamus), which passes the message to the motor cortex and tells your muscles how to adjust. Not so fast, it might caution, or a tad to the left. Without the cerebellum you might get lucky and grab your glass of wine every so often, but it’s more likely that you’d flail your arm too far one way, then correct wildly the other way and knock the glass over. In other words, the cerebellum makes grace and precision possible. It helps control the timing of movements, enabling you to walk, talk, jump, and swallow smoothly. Even some involuntary movements, like breathing, depend on the cerebellum to some extent.

  When the cerebellum deteriorates, then, your balance falters and your movements become clumsy. Hence the trembling, the eye-twitching, and the jerking gait of kuru victims. Pathological laughter can also arise when a circuit that involves the cerebellum suffers damage. And of course degenerative brain diseases rarely confine themselves to one spot in the brain. The snout and grasp reflexes, and the general cognitive decline of victims, told neurologists that kuru eventually radiated outward and affected structures like the frontal lobes.

  Even as the anatomical damage became clear, though, the underlying cause of kuru remained murky, especially on a molecular level. Some scientists jumped to the conclusion that because kuru often ran in families, it must be genetic. But as Gajdusek knew, this theory had holes. For one thing, kuru spread not only within families but also sometimes from one unrelated adult to another, which isn’t genetic behavior. Moreover, adult males almost never got kuru, while adult women did frequently. That might suggest something sex-linked—except that the incidence was equal among prepubescent boys and girls. Gajdusek suspected that kuru spread via infection. But that theory ran aground on the fact that the brains he autopsied showed no inflammation and zero other signs of infection.

  Nevertheless, the autopsies did reveal other clues. In 1957 an American colleague of Gajdusek’s discovered “plaques” in the brains of kuru victims—gnarly black burrs of protein a thousandth of an inch in diameter. The colleague also noticed a proliferation of astrocytes, a type of glial c
ell shaped like a star. Close to half the cells in the brain are astrocytes, and they play an important role in forming the blood-brain barrier, a protective sheath around blood vessels that blocks foreign material from entering the brain. But for whatever reason, astrocytes also multiply beyond control in the gray matter whenever neurons die off, eventually forming scars. The colleague had no idea what might be causing the protein plaques and astrocyte scars in kuru victims, but he did note a resemblance to Creutzfeldt-Jakob disease (a.k.a. human “mad cow” disease).

  Two years later came another clue, from across the Atlantic Ocean. After a friend recommended he visit, an American veterinarian named William Hadlow attended a museum exhibit on kuru in London. He wandered about among the Fore artifacts, interested but hardly engrossed, until some photographic blowups of kuru brains arrested him. The tissue in the pictures looked oddly spongy, and oddly familiar. Hadlow had studied scrapie, a disease that wrecks the brains (especially the cerebellums) of sheep, causing them to stagger and to scrape their skin raw on trees or fences. Some sheep even hop like rabbits. Scrapie-infested neurons have holes in them, as if tiny carnivorous moths had invaded. Scrapie brains also contain larger holes, where whole bundles of neurons have died. Hadlow noticed that kuru brains had the exact same patterns of holes—the exact same spongy appearance. He quickly wrote a paper, and Gajdusek got in touch with him shortly thereafter. As with the Creutzfeldt-Jakob link, the scrapie connection was a great lead but frustrating, since no one knew what—toxins? genes? viruses? some combination?—caused either disease.

  The words “slow down” had no meaning for Gajdusek, but with so many other scientists now studying kuru, he did decide to indulge some of his other interests, especially anthropology. He built a bamboo hut for himself in the eastern highlands and began documenting life there, taking thousands of photographs and literal miles of reel-to-reel video. Despite the constant mist and paper-curling humidity, he also filled 100,000 journal pages with field notes about most everything under the sun—local songs, etymology, bawdy gossip, recipes, the inroads made among the locals by communism and Christianity. He used the journals as a diary as well, recording how much weight he’d lost in the field (25 pounds, down to 160) and his fantasy that he could see Sputnik circling among the stars overhead.

  As a child-development specialist, one subject above all obsessed Gajdusek—sexual initiation rites—and he traveled widely across the highlands, far beyond the Fore tribes, to gather information on them. At around age seven, boys in some highland tribes moved into special domed huts, where they spent their days and nights servicing older adolescents and occasionally grown men. “You should not be afraid of eating penises,” the elders instructed them: they believed that semen strengthened young boys the way breast milk strengthened infants. Oral and anal sex also allowed the boys to “store up” semen, since some tribes believed that men didn’t produce semen naturally. Gajdusek recorded every rite he could, down to the details of who got frisky with whom. He also marveled at how some boys even “flirted” with him, batting their eyes and stroking his pasty skin. In his field notes he emphasized that the tribes sanctioned all this underage sex, and argued that it served an important social function in keeping the men from warring over women. (Other anthropologists rolled their eyes at such interpretations.) What’s more, the rites helped Gajdusek realize that the “stuffy” sexual mores of the world he’d grown up in were not universal.

  In fact the more he immersed himself in highland culture, the more Gajdusek found his past life wanting. He never renounced Western civilization entirely: in particular, he devoured the decadent literature of Henry James and Marcel Proust during his spare hours in the field. But right in the middle of some passage about dukes and duchesses, he might glance up to see Papuan youths dancing outside his hut, wearing feathered headdresses and tusks through their noses. Like Gauguin, this primitive life attracted him, and the dueling impulses—the intellectual and the primal—warred for his soul. One colleague remembered him disappearing into the bush for weeks, then stumbling into a dinner party after his journey in a dirty T-shirt and shorts, one shoe missing. However disheveled, he always dazzled guests with repartee—bouncing until 4 a.m. from Melville to meadow mice to Plato to Puritanism to suicide to Soviet foreign policy—all before disappearing into the wilderness again. Like Kurtz in Heart of Darkness, he seemed to be wrestling with all of Western civilization.

  Meanwhile, the Fore had their own bone to pick with Western civilization, particularly with Western medicine. Doctors had recently used “shoots”—injections of medicine—to eradicate leprosy in the area. Although thankful, the natives didn’t take this as a sign of the superiority of Western science; rather, they concluded that Western doctors must be powerful sorcerers indeed, far more powerful than the native sorcerers who caused diseases. So when doctors set about trying to cure kuru, Fore expectations were high. Unfortunately, none of the vitamins, tranquilizers, steroids, antibiotics, liver extracts, or other medicines that Gajdusek and company hauled into the field did any good: kuru always killed. After years of futile interventions, the Fore began to seethe. The white men took, took, took, they complained—took bodies, took blood, took brains—but gave nothing back. Even those who believed in Western medicine lashed out. One of Gajdusek’s companions fumed that he knew America had “the big microscope” capable of curing any disease, and he couldn’t understand why Gajdusek didn’t hurry the hell up with kuru.

  As the situation unraveled, the Australian government, desperate to stop kuru, considered building a giant fence around the Fore and confining them to a “reserve.” (Not only would the fence keep the Fore in, they noted, it would keep Gajdusek out.) Inspired by the genetic theory of kuru, officials also discussed sterilizing the tribe.

  But with each new victim, it became clearer that the genetic theory didn’t hold water: kuru simply spread too quickly, killing most people before they’d passed on their genes. Plus, some women who were genetically distinct from the Fore and had only married into the tribe had also come down with kuru.

  At the same time, no other possible cause made sense. Kuru was neurological, clearly. But scientists had failed to find any bacteria or viruses in the brains of victims. Other experiments ruled out hormone imbalances, autoimmune diseases, metal toxins, plant toxins, insect toxins, alcoholism, and STDs. Some doctors suggested cannibalism as a factor, but the practice had already been outlawed by then. Besides, the Fore had always cooked the bodies thoroughly before eating them, and their customs forbade children from dining on brains anyway, because eating brains supposedly stunted their growth.

  With the Fore growing testier and testier, doctors in the field resorted to bartering treasure for tissue, which made for some ugly scenes. Often the doctors would camp outside a village that had a terminal kuru case, erecting a few poles and throwing up a tarp for a makeshift autopsy clinic. At the first howl of mourning they’d enter the family’s hut and start negotiating, offering axes, blankets, tobacco, salt cakes, even American cash. One man argued that if white men took his “meat” (his wife’s brain), he should get meat in return. The doctors rustled up a three-pound ham—at which point the husband thanked them, joined the mourners outside, and wailed louder than anyone. The autopsy often took place under kerosene lamps or in drizzling rain, and it could take hours of slicing, cracking, and sawing to liberate the brain and spinal cord—an eon in a place with spotty refrigeration. Doctors wrapped the autopsy up by stuffing cotton balls into the skull and returning the body. They then had the distasteful job of making sure the villagers buried the body instead of eating it.

  As for Gajdusek, he continued his anthropologic-cum-medical work, and despite admonishing himself not to, he found himself more and more entangled with the personal lives of his patients. One sad incident involved Kageinaro, a young boy. Although frisky and “flirty” on previous encounters, Gajdusek entered Kageinaro’s village one day to find him acting aloof and distant. Gajdusek asked the boy’s friend
what the matter was. The friend sighed, “Me tink ’e gat sik.” Sick. “All at once,” Gajdusek recalled, “I knew another of my boys had kuru.” That night he insisted Kageinaro sleep beside him, for comfort; he wrote in his journal the next morning that “if kuru is contagious I certainly have it.” Gajdusek also returned months later to be with Kageinaro as he died, pulling him out of the soiled “lair” where his family had abandoned him. The boy reeked; his eyes flinched from the sunlight; he turned his crusted mouth away from Gajdusek in embarrassment. Gajdusek comforted him as best he could, holding him and giving him water. Most of it ran in rivulets down Kageinaro’s cheeks, since he couldn’t swallow. Gajdusek, mirroring the boy’s face, sobbed.

  Scientists soon entered Kageinaro’s name in The Book. This pile of white looseleaf sheets, bound together and carried in an attaché case, acted as a sort of Papuan Doomsday Book, recording every known kuru victim from 1957 forward. As a scientific document The Book is a marvel—scientists had never tracked a disease with such precision. As a social document The Book is simply sad, an unparalleled chronicle of devastation. It records that 145 of the 172 hamlets in the area lost someone to kuru, and some villages lost 10 percent of their women in one year. Reading between the lines, the entire social order was crumbling, and while the dokta bois worked and worked, hauling the brains of loved ones to patrol stations and even visiting the villages of enemies to collect samples, The Book just kept getting fatter and fatter. It eventually swelled multiple inches thick.