Questions and answers on Tourette syndrome.
A: Tourette Syndrome (TS) is a neurological disorder characterized by tics - involuntary, rapid, sudden movements that occur repeatedly in the same way. The symptoms include:
1. Both multiple motor and one or more vocal tics present at some time during the illness although not necessarily simultaneously;
2. The occurrence of tics many times a day (usually in bouts) nearly every day or intermittently throughout a span of more than one year; and
3. The periodic change in the number, frequency, type and location of the tics, and in the waxing and waning of their severity. Symptoms can even disappear for weeks or months at a time.
4. Onset before the age of 21.
The term, "involuntary," used to describe tics is a source of confusion since it is known that most people with TS do have some control over their symptoms. What is not recognized is that the control, which can be exerted from seconds to hours at a time, only delays more severe outbursts of symptoms. Tics are experienced as irresistible and, as the urge to sneeze or scratch a mosquito bite, must eventually be performed. People with TS often seek a secluded spot to release their symptoms after delaying them in school or at work. Typically, tics increase as a result of tension or stress and decrease with relaxation or concentration on an absorbing task.
Q: How are tics classified?
A: The two categories of the tics of TS and some common examples are:
Motor - Eye blinking, head jerking, shoulder shrugging, and facial grimacing.
Vocal - Throat clearing, barking noises, sniffing, and tongue clicking.
Motor - Jumping, touching other people or things, smelling, twirling about, and self-injurious actions including hitting or biting oneself.
Vocal - Uttering ordinary words or phrases, coprolalia (vocalizing socially unacceptable words), and echolalia (repeating a sound, word, or phrase just heard).
The variety or tic-like symptoms that can be seen in TS is enormous. The complexity of some symptoms often confuses family members, friends, teachers, and employers who may find it hard to believe that the actions of vocal utterances are involuntary.
Q: Do people with TS have other associated
behaviors in addition to tics?
A: Yes, additional problems may include:
Obsessive Compulsive Traits, in which the person feels that something must be done over and over. Examples include touching an object with one hand after touching it with the other hand to "even things up" and repeatedly checking to see that the flame on the stove is turned off. Children sometimes beg their parents to repeat a sentence many times until it "sounds right."
Hyperactivity and/or Attention Deficit Disorder (ADD), which occur in many people with TS. Often children will show signs of hyperactivity before TS symptoms appear. Indications of hyperactivity and ADD may include: difficulty in concentrating; failing to finish what is started; not seeming to listen; being easily distracted; often acting before thinking; shifting constantly from one activity to another; needing a great deal of supervision; and general fidgeting. Adults may have residual signs of ADD such as overly impulsive behavior and concentration difficulties.
Learning disabilities such as dyslexia, arithmetic disorders, and perceptual difficulties.
Difficulties with impulse control, which may result, in rare instances, in overly aggressive behaviors or socially inappropriate acts.
Sleep disorders, which are fairly common among people with TS. These include walking or talking in one's sleep and frequent awakenings.
Q: What are the first symptoms?
A: The most common first symptom is a facial tic such as rapidly blinking eyes or twitches of the mouth. However, involuntary sounds, such as throat clearing and sniffing, or tics of the limbs may be the initial sign. For some, the disorder begins abruptly with multiple symptoms of movements and sounds.
Q: What causes the symptoms?
A: The cause has not been definitively established, although current research presents considerable evidence that the disorder stems from the abnormal metabolism of at least one brain chemical (neurotransmitter) called dopamine. Undoubtedly, other neurotransmitters also are involved.
Q: Is it inherited?
A: Genetic studies indicate that TS is inherited as a dominant gene that may produce different symptoms in different family members. A person with TS has about a 50% chance of passing the gene to one of his/her children. However, that gene may express itself as TS, as a milder tic disorder, or as obsessive compulsive symptoms with no tics at all. It is now known that a higher than normal incidence of milder tic disorders and obsessive compulsive behaviors occur in the families of TS patients.
The sex of the child also influences the expression of the gene. The chance that the child of a person with TS will have the disorder is at least three times higher for a son than for a daughter. Yet only about 10% of the children who inherit the gene will have symptoms severe enough to ever require medical attention. In some cases TS may not be inherited, and is identified as sporadic TS because the cause is unknown.
Q: How is TS diagnosed?
A: The diagnosis is made by observing the symptoms and by evaluating the history of their onset. No blood analysis, x-ray, or other type of medical test exists to identify TS. However, a doctor may wish to order an EEG, CAT scan, or certain blood tests to rule out other ailments that could be confused with TS.
Q: Is there a cure?
A: Not yet.
Q: Is there ever a remission?
A: Some people experience either a complete remission or a marked improvement in their late teens or early twenties. Many of the people with TS get better, not worse, as they mature, and those diagnosed with TS can anticipate a normal life span.
Q: How would a typical case of TS be described?
A: The term typical cannot be applied to TS. The expression of symptoms covers a spectrum from very mild, which is true of most people, to quite severe.
Q: How is the Syndrome treated?
A: The majority of people with TS are not significantly disabled by their tics or behavioral symptoms, and therefore do not require medication. However, there are medications available to help control the symptoms when they interfere with functioning. The drugs include haloperidol (Haldol[R]), clonidine (Catapres[R]), pimozide (Orap[R]), fluphenazine (Prolixin[R], Permitil[R]), and clonazepam (Klonopin[R]). Stimulants such as Ritalin[R], Cylert[R], and Dexedrine[R] that are prescribed for hyperactivity may increase tics, and their use is controversial.
The dosage necessary to achieve maximum control of symptoms varies for each patient and must be gauged carefully by a doctor. The medicine is administered in small doses with gradual increses to the point where there is a maximum alleviation of symptoms with minimal side effects. Some of the undesirable reactions to medications are muscular rigidity, fatigue, and motor restlessness, most of which can be reduced with specific medications. Side effects that include depression and cognitive impairment can be alleviated with dosage reduction or a change of medication.
Other types of therapy may also be helpful. Psychotherapy can assist a person with TS and his/her family cope, and some behavior therapies can teach the substitution of one tic with another that is more acceptable. The use of relaxation techniques and/or biofeedback can serve to alleviate stress reactions that cause tics to increase.
Q: What is the current focus of research?
A: Since 1984, the TSA has directly funded important research investigations in a number of scientific areas relevant to TS. Recently, studies have intensified to understand how the disorder is transmitted from one generation to the next, and researchers are working toward locating the gene marker for TS. That focus has been enhanced by the efforts of a TSA supported international group of scientists who have formed a unique network to share what they know about the genetics of TS and to systematically cooperate to unravel the unknown. Additional insights will be obtained from studies of large families (kindreds) with numerous members who have TS. At the same time, investigators continue to study specific groups of brain chemicals to better understand the Sydrome and to identify new and improved medications.
Q: How many people in the U.S. have Tourette?
A: Since many people with TS have yet to be diagnosed, there are no absolute figures. The official estimate by the National Institutes of Health is that 100,000 Americans have TS. Recent genetic studies suggest that the figure may be one in two hundred if those with chronic multiple tics and/or transient childhood tics are included in the count.
Q: Do TS patients have special educational needs?
A: While school children with TS as a group have the same IQ range as the population at large, many have special educational needs. It is estimated that many may have some kind of learning problem. That condition, combined with attention deficits and the problems inherent in dealing with the frequent tics, often calls for special educational assistance. The use of tape recorders, typewriters, or computers for reading and writing problems, untimed exams (in a private room if vocal tics are a problem), and permission to leave the classroom when tics become overwhelming are often helpful. Some children need extra help such as access to tutoring in a resource room.
When difficulties in school cannot be resovled, an educational evaluation may be indicated. A resulting identification as "handicapped" under federal law will entitle the student to an Individual Education Plan (IEP) to address specific educational problems in school. Such an approach can significantly reduce the learning difficulties that prevent the young person from performing at his/her potential. The child who cannot be adequately educated in a public school with special services geared to his/her individual needs may be served best by a special school.
Q: Is it important to treat Tourette Syndrome early?
A: Yes, in those instances when the symptomatology of the condition is viewed by some people as bizarre, disprutive, and frightening. Not infrequently, it provokes ridicule and rejection by peers, neighbors, teachers, and even casual observers. Parents may be overwhelmed by the strangeness of their child's behavior. The child may be threatened, excluded from family activities, and prevented from enjoying normal interpersonal relationships. These difficulties may become greater during adolescence, an especially trying period for young people and even more so for a person coping with a neurological problem. To avoid psychological harm, EARLY DIAGNOSIS AND TREATMENT ARE CRUCIAL. Moreover, in many cases it is possible to control the symptoms with medication.
Q: What kinds of family services exist?
A: Local TSA support groups allow families to exchange ideas and feelings about their common problems. Often family therapy is helpful. Parents of a child with TS have to walk a fine line between understanding and overprotection. They are constantly faced with deciding whether or not certain actions are the expression of TS or just poor behavior. Parents then must determine the appropriate response. The child should be encouraged to control what he/she can whenever possible, and to try to substitute what is socially acceptable for what is not. Parents are urged to give their children with TS the opportunity for as much independence as possible, while gently but firmly limiting attempts by some children to use their symptoms to control those around them.
Q: How did Toureffe Syndrome get its name?
A: In 1825 the first case of TS was reported in medical literature with a description of the Marquise de Dampierre, a noble woman whose symptoms included involuntary tics of many parts of her body and various vocalizations including coprolalia and echolalia. She lived to the age of 86 and was described by Dr. Georges Gilles de la Tourette, the French neurologist for whom the disorder was named. Samuel Johnson and Andre Malraux are among the famous people who are thought to have had TS.
Q: What is the Tourette Syndrome Association?
A: TSA is the only national voluntary non-profit membership organization dedicated to:
Identifying the cause;
Finding the cure; and
Controlling the effects of TS.
Members include individuals with the disorder, their relatives, and other interested and concerned people. The Association, which was founded in 1972, develops and disseminates educational material to individuals, to professionals, and to agencies in the fields of health care, education, and government; operates support groups and other services to help people and their families cope with the problems that occur with TS; and stimulates and funds research that will ultimately find the cause of and cure for TS while, at the same time, seeking to improve medications and treatments.
* Organizes workshops and symposiums for scientists, clinicians, and others working in the field of TS.
* Maintains listings of those diagnosed with TS.
* Sponsors the Tourette Syndrome Brain Tissue Program involving brain banks on the East and West Coasts.
* Serves thousands of members throughout the USA.
* Increases the knowledge and sensitivity of health care professionals to TS through exhibits at conferences, the dissemination of literature, and the organization of national meetings.
* Develops and maintains state-by-state lists of doctors who diagnose and treat TS.
* Organizes and assists local chapters and support groups throughout the US and around the world.
* Represents the interests of members to the government on critical policy issues including orphan drugs, insurance, and employment.
Q: Why become a member of the Tourette Syndrome Association?
A: * To join other patients and families at meetings to discuss common problems and offer mutual support.
* To help effect the early identification and treatment of TS.
* To obtain, through the quarterly TSA Newsletter, the latest information on treatment, research programs, and scientific discoveries.
* To receive discounts on medications through the TSA pharmacy service.
* To obtain films, videotapes, and publications at reduced rates.
* To support TSA programs in patient advocacy.
* To become eligible for discounted registration fees at TSA National Conferences.
* TO HELP CONQUER TOURETTE SYNDROME.
The Tourette Syndrome Association has an extensive list of publications that discuss in detail many of the topics touched upon in this pamphlet. Their titles are contained in the TSA Catalogue of Educational Materials.
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|Publication:||Pamphlet by: Tourette Syndrome Association|
|Date:||Sep 23, 1991|
|Previous Article:||Facts you should know about the genetics of Tourette syndrome.|