by Oliver Sacks
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LOWELL AND I TRAVELED to Amsterdam, where we had been invited to appear together on a widely seen television show. I had fallen in love with Holland as a teenager—the place and, no less important, the sense of intellectual, moral, and creative freedom which had characterized its people since the days of Rembrandt and Spinoza. (When I first went to Holland, I was struck by the fact that the paper currency indicated its denominations in Braille as well as print.)
How, I wondered, would the Dutch see Tourette’s? Would their freedom and independence of mind reduce the shock, fear, and anger that people with Tourette’s can provoke?
The day before our television interview, we strolled around Amsterdam—I followed a few yards behind Lowell so that I could watch the reactions to his strange, sudden movements and noises. People’s reactions appeared openly on their faces as they passed by us—some amused, some disturbed, and a few outraged.
Apparently, many people saw our television interview the following day, for when we went out again the next morning, the reactions were totally different. There were smiles, looks of curiosity, and friendly greetings, as people now seemed to recognize Lowell and to understand something about Tourette’s. It drove home to us how essential it was to educate the public and transform their understanding—and how this could be done overnight, by a single television show.
That evening, relaxed, we went to a bar, where we were offered some pot and smoked it outside. We spent hours wandering around the city—looking at churches, at reflections in the canals, at shop windows, at people. Lowell, his camera at the ready, felt he was getting the best photos of his life. When we got back to our hotel later that night, and the bells of old churches started to toll, I had a sense of euphoria. Everything was right in the universe. This was the best of all possible worlds.
Lowell was less euphoric at breakfast the next morning, when he discovered that, in the happy confusion of being stoned, he had forgotten to load his camera with film, and the shots of a lifetime he thought he had captured were nonexistent.
In Rotterdam we met Ben van de Wetering, a brilliant Dutch psychiatrist who ran a clinic for patients with Tourette’s—a rarity at the time. He introduced us to two of his patients. One was a rather Teutonic young man, very formal in dress and manner, who said that he detested his Tourette’s and how it drew unwelcome attention to him. “It is completely useless!” he added, saying that he suppressed or converted his copralalia as much as possible. Thus whenever the word “Fuck!” was about to burst from his mouth, he was able to alter it, with effort, to “Frightful!” (This in fact attracted more attention than “fuck” would have done.) His Tourette’s, in response to being suppressed or sanitized in daytime, took revenge on him at night, when a litany of obscenities escaped his sleeping lips.
The other patient, a young woman, was too shy or fearful to Tourette in public—but once she was “liberated” (as she put it) by Lowell’s exuberant Tourette’s, she allowed herself to Tourette along with him, in an amazing duet of convulsive movements and noises. She told me, “There is something primal in Tourette’s—whatever I perceive or think or feel is instantly transformed into movements and sounds.” She enjoyed this rushing stream; she felt it was “like life itself,” but acknowledged that it could cause much trouble in social settings.
Tourette’s, in its effects, is never confined to the person but spreads out and involves others and their reactions; and they, in turn, exert pressure—often disapproving, sometimes violent—on those with Tourette’s. Tourette’s cannot be studied or understood in isolation, as a “syndrome” confined to the person who has it; it invariably has social consequences and comes to include or incorporate these as well. What one sees, therefore, is a complex negotiation between the affected individual and his world, a form of adaptation sometimes humorous and benign, at other times charged with conflict, pain, anxiety, and rage.
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THE FOLLOWING YEAR, Lowell and I took a road trip across the United States, visiting a dozen or so people with Tourette’s who had agreed to meet with us.
Weaving through the outskirts of Phoenix with Lowell at the wheel was a remarkable experience, as he would suddenly stamp on the brakes or the accelerator, yanking the steering wheel to one side or the other. But once we were on the open road, Lowell’s ticcy, impulsive, almost frenetic state gave way to one of stillness and concentration. He now sat calmly with his gaze fixed on the unfurling road ahead, which ran like an arrow through the central desert of Arizona. He kept our speed at an unvarying sixty-five miles per hour, never deviating.
At one point—we had been driving for three hours and needed to stretch our legs—I said to Lowell, “If you got out here and walked among the cacti, would you Tourette much?”
“No,” he said. “What would be the point?”
Lowell has strong touching tics or compulsions; he cannot be around people without having to touch them. Usually he does this gently, with a hand or a foot. It is, one feels, almost an animal urge, the way a horse butts its head against a person and nuzzles them. People’s reactions to being touched—whether positive, negative, or neutral—complete the circuit. But no such reaction could be expected from a plant.
This reminded me of a young Vietnamese man with Tourette’s I once met. He had had some coprolalia in his native land, but now that he lived in San Francisco, where few people understood Vietnamese, he no longer uttered Vietnamese expletives. Like Lowell, he said, “What would be the point?”
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SOMETIMES TOURETTERS ARE attracted by sudden bits of tactility or visual appearance—crumpledness, skewedness, odd asymmetries or shapes. (One Touretter, a wood-carver, likes to introduce sudden, convulsive asymmetries in his work—to make a chair “shaped like a tic or shriek.”) Lowell often indulges in a compulsive repetition and permutation of odd words and sounds, whose very oddness provokes and gratifies the ear. At breakfast one morning, he got excited by the oatmeal, which he called “oakmeal,” and kept repeating, “Oakmeal, oakmeal,” then, after a while, an explosive “Kkkmmm!” At another meal, he seized upon the word “lobster,” repeated “Lobbsster,” “lobbsster,” followed by “Mobbsster,” “Slobbsster,” finally concluding, “I love the sound and look of ‘bbsstt.’ ”
“I take great pleasure in repeating words over and over again,” he said. “It’s the same feeling of satisfaction as I have with my compulsive touching—like having to touch the glass on your watch, feeling the click of my nail on the glass, reveling with different senses.”
Tourette’s can be exacerbated by hunger, and when we arrived in Tucson, having driven straight from Phoenix without a food stop, Lowell was racked by such violent tics that when we entered a restaurant, every eye was drawn to him. We sat at a table, and Lowell said, “I’m going to try something. Don’t disturb me for fifteen minutes.” He closed his eyes and started to breathe deeply and rhythmically, and within thirty seconds his tics were reduced; after a minute they were totally gone. When a waiter approached—he had observed Lowell’s violent movements when we came in—I put a finger on my lips and waved him away. At exactly fifteen minutes, Lowell opened his eyes, looking very relaxed and almost tic-free. I could hardly believe it—I would have thought such a change physiologically impossible.
“What happened? What did you do?” I asked Lowell. He said that he had learned Transcendental Meditation as a way of dealing with otherwise uncontrollable ticcing in public places.“It’s just autohypnosis,” he explained. “You have a mantra, a little word or phrase repeating slowly in your mind, and you soon get into a sort of trance and become oblivious to everything. It calms me down.” He remained almost tic-free for the rest of the evening.*
Lowell had tracked down a pair of identical twins with Tourette’s in Arizona. Tourette’s had presented itself simultaneously in both boys—w
ith sudden, screeching imitations of their pet cockatoo. Subsequently, they both developed shoulder shrugging, nose wrinkling, and clicking noises, followed by complex tics and contortions of the limbs and trunk. The picture was similar but not quite identical—one had winking tics, the other had gasping tics. And yet, unless one analyzed it minutely, the two of them looked and behaved in much the same way. How much of this was some sort of genetic predisposition, I wondered, and how much their tendency to imitate each other?
We met a young man in New Orleans with severe tics as well as obsessions and compulsions—a not uncommon combination. He had once worked in a missile silo in South Dakota, a job that terrified him because, having a compulsion to fiddle with switches, he was constantly afraid he would launch a missile and set off a nuclear war. The pay was good, and all his fellow workers friendly, but the ever-present sense of risk—though exciting—undid him, so he gave the job up for something less stressful.
In Atlanta we met Karla and Claudia, another pair of identical twins, who, like Shane, had that extravagant phantasmagoric form of Tourette’s I sometimes think of as “super” Tourette’s. They were fine, funny, intelligent young women in their early twenties, their voices hoarse from incessant shouting. They had many motor tics and contortions, too, but it was through their mouths that their often bizarre impulses and fantasies broke out.
Driving with Karla and Claudia was taxing—at each turn, one would shout “Right!” and the other “Left!” They told us how they had caused stampedes in movie houses by together shouting “Fire!” and cleared beaches by screaming “Shark!” They shouted with ear-splitting loudness from their bedroom window—“Black and white lesbians!” was one such cry, and another, more upsetting, was “My father is raping me!” Although all their neighbors knew about their wild Tourette shouting, their father could never get used to it and was cruelly distressed by the girls’ shouts of “Rape!”
That our brief, patchy tour of America ended with such an extreme case is perhaps unfortunate, but such cases stay in the mind, and are often illuminating in their excess.
Lowell and I, traveling across America to meet a dozen Touretters and their families, had seen a wider range of Tourette’s than one is ever likely to see in a hospital clinic, a much wider range than an ordinary neurologist would ever encounter. If there are extravagant forms, there are also forms so mild as to escape clinical notice—Tourette’s, like autism, presents on a spectrum. One may have a very complex yet mild form of Tourette’s or a very simple yet severe form. And in any one person, Tourette’s can fluctuate in intensity, as well as in form; there may be months or years of relative remission, and months or years of cruel exacerbation.
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LOWELL HAD HEARD of an almost mythical place, in far northern Canada, where there is an entire community of Touretters, an extended Mennonite family that has had Tourette’s among them for at least six generations (he had begun to refer to it as Tourettesville). What would it mean to be a member of this huge family in which ticcing and shouting is far from unusual, almost part of a family tradition? How might Tourette’s affect or be affected by moral or religious beliefs, in such an isolated religious community? We decided to make a visit to find out.
At the airport closest to La Crete—it was little more than a landing strip in the woods—we rented a battered, splattered car, its windshield cracked from the coarse gravel on the roads. As we set off on the seventy-mile drive to La Crete, I felt the city tensions draining from me and observed Lowell’s Tourettish outbreaks growing milder, soothed by the beauty, the peacefulness, the remoteness of the countryside. Reaching the village of La Crete, we passed a Mennonite couple selling watermelons by the road. We stopped and bought one, chatted a little: they had come from British Columbia, weaving from one tiny community to another, part of the quiet, half-religious, half-commercial network that binds the Mennonite communities of the Northwest together.
The Mennonites are descendants of a large group from Germany and the Low Countries that was driven to seek religious freedom first in the Ukraine and then in Canada. They still believe in a traditional farming way of life, in closeness to the soil and to family, in nonviolence, plainness, and a partial withdrawal from the great world outside.
In La Crete, a village of seven hundred people, there is a church for each of the five main Mennonite sects, and within the order there is a considerable range of practices and beliefs. The strictest are the Old Colony Mennonites, who are suspicious of the secular in education and daily life (but even they are not absolutely secluded like the Amish, a subgroup that split off in the 1690s). These conservative villagers dress soberly in dark clothes, the women wearing headdresses—but others in the town wear jeans and shirts. There is a simplicity and down-to-earthness about the place, along with a sense of tranquillity.
This tranquillity was broken when we reached David Janzen’s house. David was the most pronounced Touretter here, and Lowell had arranged for us to meet him. Now David ran out to greet us, yelping and ticcing. The noises—ear shattering, shocking—seemed to disturb his whole being and, indeed, to disturb the whole placid face of La Crete. His cheery Tourettisms set off Lowell. They hugged each other, ticced, yelped—it was both affecting and absurd, reminding me of the excitement of two dogs meeting.
David, now in his early forties, had started having varied tics when he was eight. They were no surprise, because his mother and two older sisters also had them, as well as dozens of cousins and more distant family members. They were called “the fidgets,” and it was said that the Janzens were “restless” or “nervous.”
“Grandmother would always be blinking her eyes and smacking her lips,” said a cousin of David’s, “or clucking or hooting or making faces, whatever—it was just normal. Everybody did it.”
David’s real struggle started when he was fifteen, when he started to shout “Fuck!” in a loud voice. Such uttering of obscenities and profanities was not a common manifestation of Tourette’s in La Crete. Unlike the fidgets, the cursing smacked of a savage self or a vile prompting from the Devil. David’s compulsions started to multiply. Sometimes he had the impulse to hurt himself or break things. “Devil!” he would say to himself. “Why don’t you get out of me and leave me alone?”
The boy fled, but inward. “When I had the spell of the curse, I more or less stayed home,” he said. “I didn’t communicate with people—maybe for a year. Often at that time I just went to my room and cried myself to sleep.”
David’s parents tried to be understanding, but they, too, were confused. They saw his peculiar illness as half moral, half physical. They felt that David was subject to some outer force but also that he “allowed” the cursing. He, too, began to see himself as weak-willed. Some in La Crete had a simpler view: David was the object of divine anger and punishment. According to one villager, there was the feeling at the time that “the Janzens were strange, especially Davy. God must be punishing this family for something.”
In his early twenties, David married and started a family, though his disturbances continued. Frequently he would feel compelled to pant violently or hold his breath; these respiratory convulsions, which are not uncommon in Tourette’s, were exhausting. “I was getting so tired because I was fighting it so hard, especially if I would drive,” he recalled. He used to drive a truck from High Level to Hay River, fighting compulsions to suddenly brake or accelerate or swerve. Sometimes David would injure himself with ticcish movements. “I was once operating a chain saw and cut my leg—I know now that the movement was a Tourette,” he said, showing me the long white scar on his left knee.
David loved the hard work of farming, loved working with cattle and horses, but had difficulty because his tics startled the animals and caused them to shy. He had to give up work by the time he was thirty, but on welfare his morale sank lower and lower. Finally, at thirty-eight, David reached a crisis. “I felt I h
ad to have an answer, answers, or else I couldn’t go on.”
A local doctor had told him that he might have Huntington’s chorea, an appalling and fatal disease. In Edmonton he was told it might be myoclonus, which causes the muscles to contract suddenly. Finally David was sent to Dr. Roger Kurlan, a neurologist specializing in movement disorders at the University of Rochester in New York.
Kurlan took one look at David and said, “You’ve got Tourette’s syndrome.” David had never heard of it. As Kurlan described the tics, the compulsions, David felt immense relief. “It made me want to jump for joy,” he said. “It took away the terrible feeling of a curse. It was not the Devil working in me—which was my worst fear—and it was not medical doom. I had a simple disease, and it even had a name. A pretty name too—I kept on repeating it.”
But one point puzzled David. “Unusual, you say,” he repeated to Kurlan. “Doesn’t it run in families?”
“I rarely see it in families,” said the doctor.
“Well,” said David, a bit surprised, “most everyone I know has got Tourette’s. My family, anyhow—my mother, my two sisters.” And pulling out a pencil, he drew a family tree on the blotter, indicating more than a dozen close family members who were also affected.
When I spoke to Kurlan four years later, he told me that this was the most astonishing moment of his entire medical life. He had never considered that the syndrome could have such a strong genetic component. He made a visit to La Crete, still incredulous. He explored the village day and night for a week, interviewing a total of sixty-nine Janzen family members. Kurlan told them that what they had was neither a grave organic disease nor a curse, but a nonprogressive and probably genetically determined disorder of the nervous system.
This scientific explanation, though cause for much relief and discussion, did not entirely displace the religious view. Behind Tourette’s, the people of La Crete still saw the hand of God. But they fully embraced the term: odd behavior in La Crete is now called Touretting. Georges Gilles de la Tourette, the nineteenth-century French neurologist who identified the syndrome, would be astonished to find his name known—indeed, common currency—in a remote farming village four thousand miles from Paris.
