Tourette Syndrome

Tourette (too-RET) syndrome (TS) is a genetically ^* transmitted disorder of the central nervous system ^* associated with several kinds of motor ^* tics and at least one vocal tic ^* .

Meet Pete

One day in the middle of a math test, Pete began to make loud sniffing noises repeatedly. Other than his periodic sniffing, his sixth grade classroom was very quiet. Mr. Carlson, Pete's math teacher, put his finger to his lips, signaling Pete to be quiet. Pete imitated Mr. Carlson's gesture, putting his own finger to his lips. He repeated the gesture several times and then stopped and began making the sniffing noises again. Pete was surprised by his own behavior. He knew that he should stop, but the more he tried to stop, the more he found that he could not.

Mr. Carlson, who always understood when Pete was late with his homework, was troubled by this new classroom behavior. Pete had had difficulty finishing homework assignments and had occasionally had trouble staying still in his seat, but he had never disrupted the class before. Mr. Carlson invited Pete out into the hallway and asked if Pete would prefer to take the math test by himself. Pete said that he would. Was Pete just trying to avoid taking the math test, Mr. Carlson wondered, or might he perhaps have TS?

What Is Tourette Syndrome?

Many people with symptoms like Pete's have TS. Their tics tend to be most noticeable when they are feeling nervous, anxious (as Pete may have been during his exam), or tired. The tics tend to ease when people are focused and concentrating on something, and they are generally not evident at all when people are sleeping.

One of the many interesting features of TS is that the tics vary from person to person, in terms of the type of tic and its severity. They also may vary in the same person over time. After several months, one tic may go away and be replaced by another. It also may disappear completely. At some point in their childhood, up to one-fourth of all school-age children have simple tics, such as eye blinking, but they do not go on to have TS. Their tics simply disappear with time. Other children may experience more complex tics as time passes. Complex motor tics involve several coordinated muscle movements, such as twirling or hopping in place while walking. Complex vocal tics include babbling, echoing sounds, and babbling meaningless strings of words.

To say that a person has TS, the tics (which usually occur several times a day) must begin before a person turns 18, and they must last for more than a year. In addition, a person must have more than one motor tic and at least one vocal tic over that period of time. The tics may occur simultaneously or at different times. In rare cases, tics may involve movements that cause the person pain or injury; for example, tics that result in biting their lips or cheeks or even banging their heads. These very severe cases are the exception and not the rule in TS. More commonly, the tics involve harmless muscle movements or vocalizations, which are mild to begin with and lessen or in some cases disappear over time. The tics also may disappear for days, weeks, or years and then recur.

TS is much more common than was once believed. It is estimated that about 100,000 Americans have TS, or 1 in every 2,000 to 2,500 people. About three times that many people have other tic disorders, such as chronic ^* multiple tics or transient ^* tics. While Tourette syndrome involves a long-lasting problem with both motor and vocal tics, chronic multiple tics involve several tics that are either motor or vocal, but not both. Transient tic disorders may look like other tic disorders but are present for less than a year.

What Causes Tourette Syndrome?

Until Gilles de la Tourette, a French physician, described in 1885 the group of odd and often changing tics we now associate with TS, people like Pete often were believed to be possessed by demons. Thanks to many advances in our understanding of how information is transmitted in the brain, we now know that TS is a neurological ^* disorder characterized by chemical changes in the brain. Although the basic cause of TS and other tic disorders is still unknown, we know that TS is an inherited condition, meaning that the likelihood of TS developing in a person is passed from parent to child.

Genetics

One of the most interesting findings from studies of TS is that boys are three to four times more likely to show signs of TS than girls are. A parent with a gene for the syndrome has a one in two chance of passing it on to each child. The child who inherits the gene may show symptoms of TS or may not. In males who have the gene, there is a 99 percent chance that symptoms of the disorder will occur. In females with the gene, that likelihood is 70 percent, but girls with the gene appear to be more likely than boys with the gene to have symptoms of obsessive-compulsive disorder ^* , a condition that is believed to be linked genetically to TS.

Neurotransmitters

New research suggests that there is an abnormality in the genes affecting the brain's handling of and levels of certain brain chemicals known as neurotransmitters. Neurotransmitters carry signals from one nerve cell to another. The transmission of these signals is involved in attention and memory, movement, and moods, such as happiness, sadness, or anxiety (ang-ZY-e-tee). The neurotransmitters that may play a part in TS include dopamine ^* , serotonin ^* , and norepinephrine ^* . In this regard, TS is similar to other neurological conditions, such as Parkinson disease ^* , which are now known to be linked to specific neurotransmitter imbalances in the brain.

How Can I Tell If Someone I Know
Has Tourette Syndrome?

The short answer is that it is not always easy to spot TS. The best person to make a diagnosis of TS is a doctor who has known the person for a long time and also has gathered a detailed family medical history. Many children may have one or more simple tics, such as head turning or eye blinking, at some point in their childhood. In most cases, these tics will disappear. Besides looking for a pattern of tics that continue for a least a year, the doctor may make use of several tests, such as the Yale Global Tic Severity Scale and the Hopkins Motor/Vocal Tic Scale.

Because tics come and go, it is not unusual for a child to go to the doctor and be tic-free throughout the entire visit. Just as children may find it difficult to suppress tics at times when they may want to, they also cannot always produce tics on command. Tics tend to be more noticeable in the presence of family members and friends and less noticeable with strangers. It is important for the doctor evaluating a person for possible tics to look for related conditions and to consider the impact of the tics and related conditions on the child's ability to function in school and with friends and family.

The Tic Code (1998) stars Christopher Marquette as a boy with Tourette syndrome who dreams of becoming a jazz pianist. While playing at a local music club, he befriends a saxophone player (Gregory Hines) who also has Tourette syndrome.

Photofest

Related Conditions

It is not uncommon for people with TS to have obsessive-compulsive disorder (OCD) as well. In this disorder, a person may have recurrent, anxiety-provoking thoughts (for example, that a person forgot to lock the door when leaving home) or behavior (for example, repeatedly checking to make sure the door is locked). In the case of both TS and OCD, the person may feel unable to resist the activity, much as a person may feel unable to resist a sneeze. An important difference is that the types of behavior (compulsions) seen in OCD are typically quite complex and are voluntary, even though they may be hard to resist (like checking the lock a number of times), while those associated with TS have a more involuntary, uncontrolled quality. Even though people with TS may be able to temporarily suppress a tic, tics occur involuntarily (like blinking, sneezing, or coughing).

There are two other conditions that are commonly associated with TS: attention deficit hyperactivity disorder (ADHD) and learning disability. ADHD is a disorder associated with physical restlessness, inattention, and poor control of impulses. People with ADHD find it very difficult to organize tasks and activities, and, as a result, they are prone to move impulsively from one unfinished task to another. Physical signs and symptoms of ADHD in school-age children include squirming in their seats, fidgeting, and shaking their feet or legs excessively. In adolescents, ADHD may take the form of getting up from the table during meals, difficulty in concentrating and finishing homework, or speaking out impulsively.

Learning disabilities, such as problems in reading, writing, or math, may interfere with a person's success in school despite good intelligence. These disabilities are diagnosed when there is a difference between children's levels of achievement on academic tests and their general levels of intelligence.

How Is Tourette Syndrome Treated?

While there is no known cure for TS at this time, there are treatments that can help ease the embarrassment and discomfort associated with the condition as well as medications that can reduce the frequency and severity of the tics. Perhaps most important of all is understanding, self-understanding as well as understanding on the part of teachers, family members, and friends. It is important and helpful for all those who are involved with a person who has TS to accept that TS is a medical condition like epilepsy ^* or cerebral palsy ^* or any other neurological condition that causes involuntary responses or movements. The types of behavior associated with TS cannot be controlled, much as they might at times look or seem intentional. These types of behavior do not reflect a person's intelligence, academic ability, or potential to lead an otherwise happy and productive life.

In addition to counseling to come to terms with a diagnosis of TS, there are other measures that may help:

• Educational alternatives: Tutoring, smaller classrooms, private study areas, and alternative forms of testing (for example, oral exams for students who have difficulty writing) may help the child or teen with TS and related learning or attention disorders. In configuring the proper school environment, it is key to recognize that most children with TS have normal intelligence, but they may need special arrangements to achieve their full potential without disrupting other students.
• Relaxation and biofeedback techniques: Relaxation and biofeedback can help people with TS better manage stress that can increase the severity of the tics. With relaxation techniques, people learn to make various muscles in their bodies less tense and rigid and possibly decrease the frequency of tics. Biofeedback teaches a person to pay more attention to involuntary movements of the body, with the aim of bringing these movements under more conscious control. It is possible that relaxation and biofeedback help reduce the urge to have tics by learning to tolerate and control some of the inner tension that precedes tic behavior.
• Behavior modification: Behavior modification or habit reversal procedures also can be used to help "extinguish" or lessen disruptive behavior. Just as people with a fear of snakes can learn, with repeated exposures to snakes, to overcome their fear, people with TS can use exposure techniques to help desensitize themselves to the situations that are likely to bring about the tension that may produce tics.

Medications for Tourette Syndrome

Many people with TS may benefit from medications, particularly people with very severe and disruptive tics. Medications act on the neurotransmitters in the brain to help lessen the symptoms. Just as the tics themselves vary from person to person, the most effective medication for different people will vary, depending on other conditions they may have and how well they can tolerate the effects of a particular medication.

The medicine that has been used the longest to help minimize tics is haloperidol (sold under the brand name Haldol), a dopamine-blocking agent. The downside of halperidol and other similar dopamine-blocking agents is a possibly serious side effect called "tardive dyskinesia" (TAR-div dis-ki-NEE-zhah). Tardive dyskinesia is a neurological condition in which there are slow, uncontrollable mouth movements and drooling. Overall mental "dulling" is also common. There are other medications now available that may have fewer and less serious side effects. For example, clonidine (sold under the brand name Catapres) has proven to be effective in limiting tics and also in improving some of the attention problems of people with TS.

In addition to medications for the tics themselves, there are medications that have been used to treat related conditions, such as OCD and ADHD. As with any medications, those for TS are prescribed only after the diagnosis is confirmed. A doctor monitors a patient carefully for side effects. There may sometimes be problems with medications given to people who have more than one condition. For example, some medications that are used to treat children with ADHD can increase the severity of the tics seen in TS.

Living with Tourette Syndrome

TS is not a degenerative ^* condition that causes more damage over time. With a supportive environment and self-acceptance, the child with TS can go on to live a normal, productive life that includes not only school and work successes but also healthy friendships and relationships. In all walks of life, from sports to the arts, there are people with TS who have excelled at the activities they love. Jim Eisenreich, once a star hitter and outfielder for the Philadelphia Phillies, helped his team reach the 1993 World Series. For Eisenreich, who at one time walked off the field after twitching publicly in front of thousands of fans, the journey from shame to diagnosis, treatment, and self-acceptance paid off.