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 mirror box, however, slits the neural Gordian knot. It resolves the mismatch between the motor and sensory systems, and because the brain literally sees its commands being obeyed, it can stop sending out orders to squeeze and squeeze harder. In the sudden tranquility, the pain seeps away. To be sure, at first the relief lasts only a few hours before the phantom seizes up again. Not everyone finds relief from mirror therapy, either. But those who do, and who practice with the mirror box, can see profound improvement over time as their brain maps reorganize. In many cases the pain all but disappears. (You can think about this decoupling as the converse of neurons that fire together wire together. Here, neurons out of sync fail to link.) And in some cases the phantom itself vanishes. After Ramachandran’s first patient, D.S., drilled with the mirror box for several weeks, he felt his phantom left arm shrinking inch by inch, “telescoping” up into his shoulder. Finally, just a nub of sensation remained. Ramachandran called this the first successful amputation of a phantom limb.

  After publishing his magnum opus on phantom limbs in 1872, Silas Weir Mitchell went on to a career of such brilliance that one admirer declared him “the most versatile American since Benjamin Franklin.” He helped pioneer the study of sleep paralysis, traumatic shock, and object blindness. He also resumed his venom research; conducted some, ahem, personal experiments with hallucinogens such as mescaline; and, most infamously, invented the “rest cure” for psychological disturbances, an outgrowth of his interest in helping Civil War vets return to civilian life.

  For men, Mitchell’s rest cure consisted of a few weeks of roping cattle and sleeping outdoors in the Dakota Badlands or areas farther west. Mitchell prescribed such a retreat, with plenty of mountain air, for his buddy Walt Whitman in 1878, after tracing the poet’s dizziness, headaches, and vomiting to a small stroke. Painter Thomas Eakins also underwent this “West cure,” and the regimen supposedly cured the young Teddy Roosevelt of his effeminate voice and foppish mannerisms in the 1880s. (Before this, TR was considered soft, and people compared him to Oscar Wilde.) For women, especially for “hysterics,” Mitchell prescribed a different sort of rest cure. It consisted of six to twelve weeks of bed rest in a dark room, along with massages, electrical stimulation of the muscles, a sickening surfeit of fatty foods, and complete isolation (no friends, lovers, letters, or novels). As you can imagine, spirited women resented this. After the birth of her daughter and subsequent postpartum funk, Mitchell basically ordered the writer Charlotte Perkins Gilman to stay in bed and stop making trouble: “live as domestic a life as possible,” he said, “never touch pen, brush, or pencil again.” She responded by writing “The Yellow Wallpaper,” a classic feminist story about a woman driven mad by such treatment. (Virginia Woolf gave Mitchell a similar working over in Mrs. Dalloway.) Gilman later mailed a copy of her story to Mitchell and claimed that he amended his ways because of her, but in reality Mitchell continued to condescend to female patients, especially hysterics. When one hysteric refused his orders to end her rest cure, he threatened, “If you are not out of that bed in five minutes, I’ll get into it with you.” She held out while he removed his coat and vest, but skedaddled when he unfastened his fly. In another case, with a woman faking a mortal illness, he sent all of his assistants out of the room. When he emerged a minute later, he promised she’d be up in no time. How did he know? He’d set her sheets on fire.

  In addition to his medical practice, Mitchell began to study medical history, especially the deep and unsettling synergy between war and medicine. As he well knew, only during combat do doctors and surgeons see enough cases of ghastly things like shattered limbs to become experts on them. Moreover, the Civil War prompted great improvements in patient transport, anesthesia, and hospital hygiene. Mitchell’s general observation holds for other wars as well. Modern nursing began with Florence Nightingale in the Crimea, and the Franco-Prussian War proved once and for all the importance of vaccines. Later, the Russo-Japanese War sparked important vision research, and World War I improved the treatment of facial injuries. More recently, Korea, Vietnam, and other conflicts taught surgeons how to reconstruct mangled nerves and veins and reattach severed limbs, thus preventing phantoms from arising in the first place. And the recent wars in Iraq and Afghanistan—where close-quarter explosions left thousands of soldiers with low-level but pervasive neuron damage reminiscent of concussions—will no doubt provide their own innovative remedies. However much suffering they produce in the short term, wars have benefitted medicine profoundly.

  Even as his scholarly and scientific reputations were peaking, Mitchell felt more and more drawn to another pursuit—writing. His clinical papers on nervous ailments had always felt dehumanizing: too liable, in their pursuit of general truths, to trample an individual’s story. In contrast, fiction writing let Mitchell capture the nuances of a man’s life, and capture the way he experienced something like phantom limbs. Mitchell was actually taking part in a broader literary movement: Balzac, Flaubert, and others also poached on medical work to heighten realism and draw more convincing portraits of suffering. Nevertheless, fiction writing wasn’t deemed a respectable hobby for physicians in those days, and Mitchell’s friend (and fellow doctor and writer) Oliver Wendell Holmes Sr. advised him to keep his writing on the down low, since patients wouldn’t trust a doctor who used them as fodder.

  Only in the 1880s, after twenty years of publishing anonymously, did Mitchell come out of the authorial closet. Thereafter his scientific work tapered off, and he began writing almost full-time, eventually publishing two dozen novels. He often saddled his characters with seizures, hysteria, split personalities, and other nervous ailments. And although he wasn’t above tossing in a ghost to enliven the plot, he wrote mostly realistic works with an emphasis on moral dilemmas. Teddy Roosevelt declared Mitchell’s bestseller Hugh Wynne: Free Quaker probably the most interesting novel he’d ever picked up. And toward the end of his life, at age seventy-five, Mitchell finally owned up to writing “The Case of George Dedlow” four decades earlier. Mitchell had taken Dedlow’s name from a jeweler’s shop in a Philadelphia suburb, mostly because he found it apt (“dead-low”) for a double leg amputee. He’d sent the story to a female friend for feedback. Her father, a doctor, read about phantom limbs with fascination, and forwarded it to the editor of The Atlantic Monthly. Mitchell claims he forgot about the story until the page proofs and an $85 check arrived in the mail. Regardless, the story’s success galvanized him. At that point he hadn’t published anything academic about phantom limbs, and without the public outpouring for Dedlow he might never have pushed his fellow doctors to take phantom limbs seriously.

  *

  A friend once noted of Mitchell that “every drop of ink [he wrote] is tinctured with the blood of the Civil War.” Even on his deathbed—in January 1914, as the world prepared for a new war in Europe—Mitchell’s mind could only drift back to Gettysburg and Turner’s Lane. He in fact spent his last, delirious moments on earth conversing with imaginary soldiers in blue and gray, pursuing phantoms to the end.

  CHAPTER SIX

  The Laughing Disease

  So far we’ve mostly considered one-way communication—from brain to body, for example. But the nervous system also uses feedback loops, to tweak commands on the fly and combine signals in new and sophisticated ways.

  Toward the end the victims laughed frantically, explosively, on the slimmest pretense, laughed so hard they’d fall over and sometimes almost roll into the fire. Until that point their symptoms—lethargy, headaches, joint pain—might have been anything. Even when they began to stumble about and had to flail their arms in a herky-jerky dance to stay balanced, even those tics might be explained away as sorcery. But laughing could only mean kuru. Within months of the first symptoms, most kuru victims—predominately women and children in eastern Papua New Guinea—couldn’t stand upright without clutching a bamboo cane or stake. Soon they couldn’t sit up on their own. When terminal, they’d lose sphincter control and the ability to s
wallow. And along the way, many would start to laugh—laughing reflexively, senselessly, with no mirth, no joy. The lucky ones died of pneumonia before they starved. The unlucky ones were whittled down until their ribs pushed through their skin, and the women’s breasts hung deflated.

  After a few days of mourning, the local women raised the victim on a stretcher of sticks and bark, and gathered in a secluded bamboo or coconut grove distant from the men. Silently, they started a fire and greased themselves with pig fat to protect against the insects and the nighttime cold of the mountain highlands of Papua New Guinea. They laid the body on banana leaves and began sawing each joint, fraying the cartilage with rock knives. Next they flayed the torso. Out came the clotted heart, the dense kidneys, the curlicue intestines. Each organ was piled onto leaves, then diced, salted, sprinkled with ginger, and stuffed into bamboo tubes. The women even charred the bones into powder and stuffed that into tubes; only the bitter gallbladder was tossed aside. To prepare the head they burned the hair off, gritting through the acrid smell, then hacked a hole into the skull vault. Someone wrapped her hands in fern leaves and scooped out the brains and filled still more bamboo. Their mouths watered as they steam-cooked the tubes over warm stones in a shallow pit, a cannibalistic clambake. In dividing up the flesh, the victim’s adult relatives—daughters, sisters, nieces—claimed the choicer bits like the genitals, buttocks, and brain. Otherwise, people shared most everything, even letting their toddlers partake in the feast. And once they started feasting, they kept stuffing and stuffing themselves until their bellies ached, taking leftovers home so they could binge again later.

  The tribe never named itself, but explorers called them the Fore (For-ay), after their language. In Fore theology, consuming someone’s body allowed his or her five souls to enter paradise more quickly. Moreover, incorporating their loved ones’ flesh into their own flesh comforted the Fore, and they considered this more humane than letting maggots or worms disgrace someone. Anthropologists noted another, more prosaic reason for the feasts. For food, the Fore mostly gathered fruits and vegetables and scraped a few kaukau (sweet potatoes) out of the poor, thin mountain soil. A few villages kept pigs, and hunters speared rats, possums, and birds, but the men usually hoarded these spoils. The funeral feasts let women and children gorge on protein, too, and they especially enjoyed eating kuru victims. Kuru left people sedentary, unable to walk or work, and those who died of pneumonia (or were euthanized by smothering before they’d starved) often had layers of fat.

  Despite the feasts, kuru—from a local word for “cold trembling”—alarmed the Fore, and they concealed its existence from the outside world for decades. Doing so wasn’t hard, as they lived in the eastern highlands of New Guinea, among the most isolated places on earth; through the mid-1900s, many tribes there didn’t know salt water existed. But soon enough the outside world began wrapping its coils around the Fore and other nearby groups. Gold miners tramped through the highlands in the 1930s, and a Japanese plane crashed there during World War II. Missionaries dribbled in, and in 1951 Australia established a patrol post for men who enjoyed wearing short khaki shorts and pointing rifles at people who lacked even metal tools. Kuru had reached epidemic levels by then, but most of the outsiders were worried about other things, like the tribes’ excessive violence and their outré sex habits. (One-quarter of adult males in the highlands died in raids or ambushes, and some tribes initiated boys into manhood with ritual sodomy.) Some white visitors did catch a glimpse of a kuru invalid being hustled out of sight now and then, or noticed the curious lack of burial grounds in a place with such high mortality. But even the first western doctor to examine a kuru patient arrived at the rather Victorian diagnosis of hysteria, hysteria fueled by colonialism and the erosion of traditional tribal life.

  The more cases of kuru that emerged, though, the more empty that diagnosis seemed. How could a seven-year-old with no memories of tribal life come down with hysteria, much less die of it? Kuru was clearly organic, and the movement and balance problems suggested brain trouble. But whether kuru was genetic or infectious, no one knew. To compound the mystery, unlike all other known infections or neurodegenerative diseases, which don’t discriminate by race or creed, kuru attacked only the Fore and their neighbors, some 40,000 people; The Guinness Book of World Records once named kuru the rarest disease on earth. But exactly because of its oddities, this rarest of diseases soon became a global obsession, with samples of Fore brains speeding across the globe and opening up whole new realms of neuroscience.

  The highlands attracted a strange breed of visitor. People who laughed off leeches and lice. People who didn’t mind that the natives greeted them by fondling their breasts or sprinkling pig’s blood on them. People who shrugged when the roads were washed out yet again and didn’t blink when told that reaching a village a few miles distant would require an eight-hour hike around gorges and cliffs. You almost had to thrive on hardship, and throughout the 1950s New Guinea attracted its share of misfits—none more misfit than D. Carleton Gajdusek.

  Born to a butcher in New York State, Gajdusek (GUY-duh-sheck) proved a science prodigy as a boy. He sailed through school, and on the stairs leading up to his lab in the attic he painted the names of Jenner, Lister, Ehrlich, and other great biologists. (A dubious legend had it that he left the top stair blank, for himself.) Still, he had trouble relating to his peers, to say the least; he once threatened to poison his entire class with the cyanide his aunt had given him to collect bugs. So at age nineteen this young man with icy blue eyes and pitcher ears ran off to Harvard Medical School, where he earned the nickname Atom Bomb for his intensity. He specialized in pediatrics, then did graduate work in California on microbes. His circle of colleagues there included James Watson.

  Neuroscientist and adventurer Carleton Gajdusek. (National Library of Medicine)

  But just as Gajdusek began to establish himself in American science, he began to chafe at the conventions of bourgeois American life. He finally escaped under the auspices of the army medical corps and set about wandering through Mexico, Singapore, Peru, Afghanistan, Korea, Turkey, Iran. At each stop he hunted out children with rabies or plagues or hemorrhagic fevers, doing pathbreaking work on little-known diseases. He made friends easily and lost them even more easily, often in blowout fights. In fact he had little personal life beyond his pediatric work: a colleague once observed that he had “no interest in women, but an almost obsessional interest in children.” Like the Pied Piper, he attracted a coterie of boys in every remote village, and he once wrote in his journal, “Oh, that we might be Peter Pans and live always in Never-Never Land.”

  In early 1957 he visited New Guinea, planning to cruise right through—until he heard about kuru. Kuru combined his interests in microbiology, neurology, children, and remote cultures, and the colleague who first briefed Gajdusek about it compared his reaction “to showing a red flag to a bull.” Gajdusek caught the next bush plane up to the highlands and began tramping from village to village over some of the steepest, slipperiest terrain on earth. He quickly memorized the symptoms—twitching eyes, a staggering gait, trouble swallowing, laughter—and identified two dozen kuru victims within a week, sixty within a month. With growing excitement he also began writing letters to colleagues, alerting them to this new disease.

  He spent the next few months conducting a kuru census, visiting every village he could and taking tissue samples from victims. To this end he recruited—with soccer balls and other toys—an entourage of ten-to thirteen-year-old dokta bois (doctor boys), dozens of whom might accompany him on a patrol. They marched for hours with Gajdusek every day, clad in white laplaps (waistcloth skirts) and carrying boxes of rice, tinned meats, and medical supplies on poles over their shoulders. They had to dodge bees and mudslides and stinging plants. They made tea in streams and wielded bamboo torches after dark. Their shelters for the night were often barely distinguishable from the surrounding shrubbery, and they lived in perpetual fear of ambushes from neighbors with bows
and arrows. Reaching some villages required crossing gorges on bamboo bridges that disintegrated with each step, the chaff flaking off and floating down a hundred feet to the rivers below. Naturally, most boys saw the patrols as grand adventures, the happiest hours of their lives.

  At every stop Gajdusek asked about kuru, and the more enterprising dokta bois snuck into the bush to rustle out victims who’d been hidden away. Some boys were beaten for this by family members who wanted their mothers and aunts and children to die in peace. But whenever a victim agreed, Gajdusek took blood and urine samples in makeshift bamboo tubes and packed them away in his supply boxes.

  Two young kuru victims. (Carleton Gajdusek, from “Early Images of Kuru and the People of Okapa,” Philosophical Transactions of the Royal Society B 363, no. 1510 [2008]: 3636–43)

  After a thousand miles of hiking, Gajdusek had determined just how bad things were. Roughly 200 people were dying of kuru annually, the proportional equivalent of 1.5 million U.S. deaths every year. And things were actually worse than that sounds. Because kuru targeted women and children, it threatened to extinguish the Fore culture, since the younger generation couldn’t replenish itself. More acutely, the chronic shortage of women, a common cause of war among hunter-gatherers, seemed likely to ratchet up tensions even more.