It's Nobody's Fault

by Harold Koplewicz

  “TWITCH AND SHOUT”

  When I was a kid, I used to love talking to my best friend’s father, partly because he was a terrific guy but also because he did what I thought was a great imitation of a turtle—craning his neck and bobbing his head all the time. He was constantly in motion, and quite noisy too; I’ll always associate the sound of keys and money jangling in a pocket with my childhood and Mr. Knepper. Sometimes the sound was so loud I couldn’t hear his voice, especially since he was always clearing his throat. My parents told me that Mr. Knepper was “a very tense man.” My guess is that my pal’s dad had a mild case of Tourette syndrome.

  Over the last several years Tourette syndrome (TS), a relatively rare brain disorder characterized by involuntary motor and vocal tics that begin before the age of 21 and last for at least a year, has been getting a lot of attention, in fact and fiction. A story line of the hit TV show LA Law had one of the Mackenzie, Brackman attorneys defending the rights of a man who’d been fired because of Tourette syndrome. Oliver Sacks wrote An Anthropologist on Mars, a book about Dr. Carl Bennett, a Canadian surgeon diagnosed with TS at age 37. The subject even made a couple of appearances on the sports pages, when Phillies outfielder Jim Eisenreich and Denver Nuggets guard Mahmoud Abdul-Rauf went public with the news that they have TS. In the spring of 1995 a documentary about Tourette syndrome—called Twitch and Shout—won critical acclaim.

  The TS numbers aren’t very large. It is estimated that some 200,000 Americans have full-blown Tourette syndrome and that another 1.8 million have some tics. Only about 1 in 2000 schoolchildren have the disorder, but as many as 15 percent have transient tics, ones that come and go. TS is much more commonly diagnosed in males; some studies say that the ratio of males to females is 3 to 1, while others put it at 5 to 1.

  It often takes some time for tics to make an appearance in a child later diagnosed with TS, although very young children will sometimes rock or bang their heads. Most kids with TS were being treated by age two or three for sleep disorders, language problems, or behavioral difficulties; parents say they’re restless, difficult, or oppositional—like the firebug Kevin, described earlier in this chapter. They’re easily frustrated and have frequent temper tantrums. They have more sleep disorders, especially sleepwalking, than the general population of youngsters. They usually have trouble concentrating on their studies and getting along with their peers, so teachers may think that these children have attention deficit hyperactivity disorder. By the time these kids reach my office, many of them have been examined by a battery of experts, from allergists to neurologists to ophthalmologists. The words “Tourette syndrome” probably have not come up. If the words have been spoken, there’s a good chance the parents have not accepted them as gospel.

  It’s only at around age six or seven that children with TS start getting simple motor tics; the vocal tics begin at about nine. Typically the symptoms increase as a child matures, and TS is usually at its most severe during adolescence. Their symptoms may decrease, or even disappear, as kids reach their twenties, but some will continue having severe TS symptoms as adults. The worst tic I ever witnessed was in a 30-year-old man with a whole body tic; he would arch his entire body and throw himself backward.

  Motor tics seem to follow a head-to-toe progression. Someone who has had TS for a number of years will usually say that it all started with a facial tic—the eyes or nose or mouth—and then moved, in turn, to the shoulders, arms, and legs. As the disease progresses, the tics also become more complex; tics wax and wane and change form. Motor tics do not automatically indicate TS. A diagnosis of TS requires both motor and vocal tics. That’s why the documentary is called Twitch and Shout.

  THE SYMPTOMS

  The motor and vocal (also called phonic) tics associated with TS may be simple or complex. The most common simple motor tics are blinking, shrugging, grimacing, and nose twitching. The complex motor tics are slower and may appear purposeful: kissing, pinching, sticking out the tongue, touching, gyrating, throwing things, making obscene gestures (called copropraxia), and imitating the gestures of others (echopraxia). Simple vocal tics are meaningless sounds and noises, including grunting, tongue-clicking, hooting, and clearing of the throat. Complex vocal tics are words that have some meaning even if they make no sense: “But! But!” or “Oh, boy!” for example. The best known complex vocal tic associated with TS is actually the least common: coprolalia, or outbursts of foul language. (Not surprisingly, the LA Law character with TS had this symptom. That’s show biz.) Only about 15 percent of those diagnosed with TS have such outbursts. Somewhat more common is echolalia, the mimicking of the words of others.

  There’s a lot of shame connected with TS; after all, the symptoms can seem pretty strange. When kids are very young, with only minor tics, they’re usually not too anxious about it, but when the teasing starts—and it nearly always does—a child quickly loses confidence and self-esteem. Adolescence can be brutal, as a child’s symptoms typically worsen and peers become relentless in their criticism. One teenage girl I treated for tics was called a “retard” and a “mutant” by her classmates at school. That was on the good days.

  It’s easy to understand, therefore, why some children are not always forthcoming or honest about their symptoms. In fact, their imaginations sometimes work overtime as they try to invent plausible explanations for inexplicable behavior. A little boy told me he clears his throat all the time because he’s got a tickle in his throat. “Maybe it’s postnasal drip,” he said. A child who is obviously involved in an involuntary action will sometimes try to make it seem voluntary, converting a shoulder shrug into a stretch, for instance. Jessica, an 11-year-old girl I treated last year, had so many body tics she practically tied herself in knots trying to hide them. Between the energy used up by the tics themselves and the additional energy expended to camouflage them the poor kid was completely spent.

  Complicating the detection of symptoms further is the fact that in addition to waxing and waning, tics may occasionally be suppressed, either willfully or not. Suppression of tics nearly always occurs when kids are asleep or when they’re engaged in an activity that requires serious concentration. I’ve seen children with very severe tics who are tic-free when they play Nintendo. I once watched two 13-year-old boys play a killer game of handball. The boys were evenly matched, and the competition was fierce. Both kids appeared to be completely normal. When the game was over, I noticed that one of the boys—the winner, as it happened—was a mass of tics: blinking, grimacing, and shaking his head from side to side. The other boy asked him—with all the delicacy and sensitivity for which children are famous the world over—what the hell he was doing. “Oh, I just do this between games,” the boy with the tics replied.

  It’s not uncommon for a parent to notice TS symptoms while a teacher is unaware of them. If they can manage it, many kids hide their tics while they’re at school. A child will be mild-mannered and well behaved all day in class and a total mess the minute he gets home. “He’d open the door, and his books would just go flying,” said the mother of a 13-year-old boy who kept his tics under control all day at school. “One day he walked in, twitching like mad, and called me a bitch. Keeping everything bottled up all day just made everything worse. He literally couldn’t control himself for another minute. He had to let it out.” I had occasion to see this same boy at school one day. Laughing with his friends, who obviously found him very good company, he seemed like a normal all-American boy.

  THE DIAGNOSIS

  Motor and vocal tics are the essential symptoms of Tourette syndrome, but it’s rare to find a child who has just a tic disorder. Some 40 percent of all kids with TS also have attention deficit hyperactivity disorder (see Chapter 7), and even more of them—perhaps as many as 80 percent—have symptoms of obsessive compulsive disorder (Chapter 8). Many children have all three disorders at once, a situation that can, of course, cloud a diagnosis and make treatment quite complicated. The diagnosis of Tourette syndrome comes only after taki
ng a history from the child, the child’s parents, and, whenever possible, the child’s teachers.

  Ron, 10 years old, was referred to me after being diagnosed with severe ADHD and some serious language problems. During our first visit I didn’t notice that he had tics, although it was obvious he had a number of compulsive symptoms. For example, he had to touch everything in my office five times; I could hear him counting the touches under his breath. The following week I was talking to Ron’s mother while Ron waited outside, and I heard a barking noise. The sound was repeated several times. “That’s Ron,” his mother said finally. “You know, he does this all the time.” His vocal tic was later confirmed by his teachers, and it became clear that he had some motor tics as well. His family history was full of language disorders, stuttering, and tics. In addition to ADHD and OCD Ron obviously had Tourette syndrome.

  Janis’s parents brought her in for an examination because her teachers had been complaining about her behavior in school. Mom and Dad suspected ADHD. Janis, nine years old when I first saw her, had had what her parents called “crazy fears” nearly all her life. Her earliest fear was of ducks. Lately she was terrified to go anywhere in the car because she thought people in the other cars were looking at her. Vans in particular sent her into a tailspin. (Janis knew, by the way, that her fears had no basis in reality, but they still caused her great distress.) This time I noticed a tic right away, since Janis had echolalia; she repeated just about everything anybody said, often many times over. Again there was a family history of tics, and Janis’s parents, when questioned directly about it, remembered that their daughter had had both vocal and motor tics in the past that had since disappeared. I diagnosed TS.

  Parents are not likely to welcome the news that a child has TS, but in my experience children are relieved to learn that what is wrong with them has, finally, a name. “What you have is called Tourette syndrome. About 200,000 other people have it too, and this is what we’re going to do to make you feel better” feels a lot more comforting to a kid than the notion that he can’t control his own body. “I can’t keep my leg from moving,” a third grader said to me with tears in her eyes. “I can’t stop blinking,” said another, equally distressed. Almost without exception kids are reassured to know that there’s a real problem and that help is on the way.

  THE BRAIN CHEMISTRY

  There’s a great deal of evidence to suggest that dopamine is the neurotransmitter that is most strongly affected in Tourette syndrome, but norepinephrine and serotonin seem to play major roles as well. (Neuro-transmitters in the brain do not act independently; they all interact.) Basically, a child with TS has too much dopamine and too little norepinephrine and serotonin. We know that medications that block dopamine and medications that increase norepinephrine are helpful in the treatment of TS. We also know that dopamine-enhancing agents make tics worse. Neuroimaging techniques, such as CAT and PET scans, have demonstrated differences in the size and activity level of certain parts of the brains of patients with TS.

  Tourette syndrome runs in families, but it’s not always a simple matter to detect the disorder. While TS is genetically transmitted, having the gene makes a person a “carrier”; it doesn’t necessarily mean he will have the symptoms of the disease. It’s extremely important to obtain a comprehensive family history when we suspect TS. Of course, not all parents realize that they have a history of tics in the family. Every child and adolescent psychiatrist has stories about parents who are less than candid in their responses, either because they’re hiding something or because they’re ignorant, blissfully or otherwise.

  Many parents who see tics in their children don’t recognize their own tics or identify them as such; they’re just harmless “habits.” One of my favorite experiences in that category came a few years ago. I was evaluating a little boy with the symptoms of TS, and I asked his parents the standard question: “Is there anyone in the family who has tics?” Both replied with an emphatic no. The problem was, at the time Dad was twitching and shrugging so much he was making the chair shake, and Mom was constantly clearing her throat.

  THE TREATMENT

  I’ll never forget the day I told Barry, a nine-year-old boy with a sweet smile and a wonderful disposition, that he had a tic disorder. “A tic disorder?” he said. “Does that mean I have to wear a flea and tick collar?”

  Nothing would have made me happier than to say yes. All doctors fantasize about a magic cure for disease. Why not a tic collar for treating Tourette syndrome? Unfortunately, however, there is no cure, magic or other, for TS. The best we can do is to control the disorder, and there are two basic methods of doing this. The first is relaxation techniques, especially self-hypnosis. The more tense and anxious a child is, the more severe his TS symptoms will be. If we can relieve stress, we can decrease symptoms. Even if the first element of the TS treatment is effective, we will almost always need the second part as well: medication. The recommended drugs for the treatment of TS fall into two major categories, neuroleptics and antihypertensives. Both offer good news and bad news.

  The neuroleptics work, but the side effects associated with them are often troublesome, so the first-line drugs for TS are the antihypertensives, especially Catapres. After a 12-week trial of Catapres most children with tics are 60 percent improved and experience limited, harmless side effects —mostly drowsiness (usually only during the early stages of treatment) and dry mouth. The Catapres doesn’t interfere with learning or general functions. Another antihypertensive that is prescribed for TS is Tenex. Klonopin, an antianxiety agent, has also been effective in suppressing tics in some kids with TS.

  The neuroleptics, most notably Haldol, Orap, and Prolixin, are better than Catapres at getting rid of tics—studies have shown 70 to 80 percent improvement in kids—but the side effects can be numerous and unpleasant: drowsiness, weight gain, decreased concentration, and sometimes impaired memory. Long-term use of neuroleptics may lead to tardive dyskinesia—involuntary muscle movement—in adults, but this effect of the neuroleptics on children has not been documented. I prescribe neuroleptics only when tics are causing great distress and dysfunction.

  Many children diagnosed with TS also are being treated for ADHD and OCD. Attention deficit hyperactivity disorder usually calls for a psychostimulant, such as Ritalin; and OCD is ordinarily treated with an antidepressant, such as Anafranil or Prozac. All these medications have possible nuisance side effects. Anafranil may cause dry mouth, drowsiness, low blood pressure, dizziness, and constipation. Prozac may cause sleepiness or lack of inhibition. Ritalin may cause tics to increase. Because of all these variables, the child’s treatment package here must be monitored especially carefully.

  It is estimated that 20 to 30 percent of all children with TS outgrow the disorder naturally in their teens or early twenties. I know of a couple of cases in which the tics just disappeared, as if by magic. One of those children has been tic-free for more than five years now. However, it would be a grave mistake for any parent to count on or even hope for a spontaneous remission. The treatment for Tourette syndrome is not ideal, but it is essential. There are risks attached to the medication, but leaving TS untreated poses a much greater risk to a child’s health and well-being.

  TS can be a very debilitating disorder, hard on the body as well as the mind. I’ve never seen a 14-year-old look as tired as Wendy. She came in with a severe blinking problem. It started when she was seven years old, but it’s gotten a lot worse lately, so bad, in fact, that it’s interfering with her ability to study. A straight-A student since first grade, she was having trouble academically. As I talked to Wendy about her problem, I discovered that blinking was just the tip of the iceberg. Many other tics were filling her day. She’s been barking, shrugging her shoulders, and twisting her upper body, sometimes hundreds of times a day. Embarrassed and ashamed about her tics, she’s been avoiding school; that is why her grades have dropped. These tics take a tremendous amount of concentration and energy. As anyone who saw the dark circles under th
is teenager’s eyes could easily tell, Wendy was exhausted.

  Children with TS who don’t receive prompt treatment will suffer in other ways too. They’ll probably be alienated by their peer group and find it difficult to function socially. TS will affect their ability to date, to marry, to go out and get a job. Not surprisingly, a tic disorder can have a devastating effect on a child’s self-esteem. Left untreated, it may contribute to depression. The worst case I’ve come across was an 18-year-old boy who, I learned, had untreated tics since he was in the first grade. By the time I saw him, he was demoralized, hopeless, even suicidal. “I’m not living. I’m just surviving,” he said in desperation. “Wouldn’t it be great not to be alive at all? Then I wouldn’t have to worry about doing all these crazy things anymore.”

  Tourette syndrome cannot be cured, true, but it can and must be brought under control with medication. With the right treatment children and adolescents with TS can lead well-rounded, productive, happy lives. Some of them even make it to the major leagues.

  PARENTING AND TS

  “We have three children, but one of them, Bradley, takes up 90 percent of our time. Brad runs all our lives. The family can’t function because we have to concentrate on making sure he’s all right all the time. When he was young, he was afraid of everything. Now that he’s older, he’s angry all of the time, always having tantrums. Every small thing in life gets blown up to a huge thing. He feels as if everyone is judging him. My husband and I have to think about him 24 hours a day. We don’t even have time to feel guilty about neglecting the other two kids.”

  Even in the best of times, parenting is a precarious balancing act—nurture and support on one side, control on the other. Maintaining the correct balance between the two is a goal that every parent takes very seriously. When a child is sick, the rules of parenting sometimes have to be revised, if not rewritten, and accommodations must be made. Still, balance—between protecting a child and helping him to get well—remains something to strive for, tics or no tics.