Tourette syndrome (TS) is an inherited neurological disorder that typically appears in childhood. The main features of TS are repeated movements and vocalizations called tics. TS can also be associated with behavioral and developmental problems.
Tourette syndrome is a variable disorder with onset in childhood. Though symptoms can appear anywhere between the ages of two and 18, typical onset is around age six or seven. Tics, which may be motor or vocal, tend to wax and wane (increase and decrease) in severity over time. Facial tics, such as rapid blinking or mouth twitches, are the most common initial sign of TS. Other early symptoms include involuntary sounds such as throat clearing and sniffing, or tics of the limbs. Symptoms usually intensify during teenage years and diminish in late adolescence or early adulthood. Patients may also develop co-occurring behavioral disorders, namely obsessive-compulsive disorder (OCD), attention deficit hyperactivity disorder (ADHD) or attention deficit disorder (ADD), poor impulse control, and/or sleep disorders. Though some children have learning disabilities, intelligence is not impaired. TS is not degenerative and life span is normal.
Tourette syndrome is classified by the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition Text Revision (DSM-IV-TR) as a "Tic Disorder." The International Classification of Disease and Related Health Problems, Tenth Revision (ICD-10) calls TS a "combined vocal and multiple motor tic disorder (de la Tourette's syndrome)." A French neurologist , Jean Marc Itard, described the first known case of Tourette syndrome in the 1825. He had recorded the ticcing and cursing behavior of an aristocratic woman, Madame de Dampierre. The disorder is named for another French physician, Georges Gilles de la Tourette, who reported a series of cases in 1885, the primary example of which was the marquise. Tourette syndrome may also be referred to as Gilles de la Tourette syndrome (GTS).
Tourette syndrome occurs worldwide, in people of all racial and ethnic groups. It is thought that approximately 200,000 people in the United States have TS. About three-quarters of patients are males. Once thought to be a rare disorder, TS is one of the most common genetic conditions. Recent estimates of prevalence suggest that TS occurs in one in 1,000 to one in 100 male children. One report indicated that prevalence may be as high as 25% in children in special education classes.
Causes and symptoms
Genetic factors are believed to play a major role in the development of TS. Several chromosomal regions have been identified as possible locations of genes that confer susceptibility to TS. Some family studies have indicated that TS is inherited in an autosomal dominant manner. In an autosomal dominant condition, an individual has a 50% chance to pass the gene to his or her children. Not everyone who inherits a TS gene will show symptoms. Approximately 70% of females and 99% of males with a TS gene will express symptoms. An individual who inherits the TS gene may develop TS, a milder tic disorder, obsessive-compulsive disorder (OCD) without any tics, or no signs of TS. The gender of a person influences the expression (the disease symptoms and severity) of the TS gene; males are more likely to have TS or tics and females are more likely to have OCD. Approximately one in ten children who inherit the TS gene from a parent will show symptoms that are severe enough to warrant medical treatment.
Non-genetic factors are also believed to contribute to the development of TS. In about 10-15% of cases, TS is not genetic. Certain stressful processes during gestation (pregnancy) or at the time of birth may increase the chance for a person to develop TS. For example, it is known that when both twins have TS, the twin who weighed less at birth tends to have more severe tics. Other non-genetic factors that may predispose a person to TS include: severe psychological trauma, recurrent daily stresses, extreme emotional excitement, PANDAS (pediatric autoimmune neuropsychiatric disorder with streptococcal infection), drug abuse, and certain co-existing medical or psychiatric conditions. In PANDAS, children experience an abrupt onset of TS symptoms and/or obsessive-compulsive symptoms following a strep throat infection.
It is thought that TS is the result of abnormal metabolism of a neurotransmitter (a chemical in the brain that carries signals from one nerve cell to another) called dopamine and possibly of other neurotransmitters including serotonin and norepinephrine. As of December 2003, the exact mechanisms by which the TS gene or genes lead to disease symptoms were unresolved. It is hoped that locating the gene or genes responsible for TS will improve understanding of how TS develops and eventually will lead to more effective treatments.
Tics seen in patients with TS can range in intensity, frequency, duration, type and complexity. Although there is wide range of severity observed in TS, the majority of cases are mild. A minority of patients has symptoms that are severe enough to interfere with daily functioning. In the most severe cases, patients experience numerous debilitating tics during all waking hours. Tics usually occur in "bouts" with many tics over a short interval of time. Many patients experience waxing and waning (fluctuations in severity) of their tics over the course of weeks or months. Tics can be made worse by stress or fatigue and tend to improve when the individual is absorbed in an activity or task that requires concentration. Although the tics associated with TS are involuntary (not deliberate), people with TS can sometimes control their tics for a period of time ranging from minutes to hours. However the tic must eventually be expressed and will come out. Coprolalia, a sensationalized type of tic in which people make obscene or socially inappropriate comments, is present in less than 15% of TS patients.
Tics are classified as either simple or complex. Simple tics are sudden, repetitive movements that involve a limited number of muscle groups. Simple motor tics are fast and without purpose. They can cause both emotional and physical pain (such as head jerking or jaw snapping). Simple vocal tics are meaningless sounds or noises. Complex tics are coordinated patterns of stepwise movements that involve multiple muscle groups. Complex motor tics appear slower and more deliberate than simple motor tics. Complex vocal tics involve meaningful words, phrases or sentences.
SIMPLE MOTOR TICS
- blinking eyes
- jerking head
- shrugging shoulders
- facial grimacing
- rolling eyes up
- smacking lips
- jaw snapping
SIMPLE VOCAL TICS
- throat clearing
- tongue clicking
COMPLEX MOTOR TICS
- touching other people or things
- twirling about
- thrusting of arms, groin, or torso
- fiddling with clothing
- self-injurious actions including hitting or biting oneself (rare)
COMPLEX VOCAL TICS
- uttering words or phrases out of context
- repeating words or sounds
- repeating others' words (echolalia)
- repeating one's own last word or sound (palilalia)
- talking to oneself
- vocalizing socially unacceptable words (a rare tic called coprolalia)
In addition to tics, patients with TS can also have additional problems that include:
- Obsessive-compulsive disorder (OCD). OCD is a condition characterized by the presence of obsessions (persistent involuntary thoughts, images or impulses that are experienced as unwanted and bothersome) and compulsions (the actual behaviors that are performed over and over in response to the obsessions). Examples of obsessive-compulsive behavior include excessive hand washing and repeatedly checking to see that a door is locked. In patients with TS, onset of OCD usually occurs before puberty and it may lead to serious impairment. It is thought that some forms of OCD have the same etiology (cause) as TS. Obsessive-compulsive behaviors can negatively impact a child's performance at school if they are time-consuming or distracting.
- Attention deficit disorder with or without hyperactivity (ADHD or ADD). Attention deficit disorder may precede symptoms of TS. It is estimated that ADD or ADHD occurs in as many as 75% of individuals with TS. Children with ADHD can be fidgety, have a very short attention span, be impulsive, and have difficulty completing tasks. ADD is similar except without the high level of activity seen in ADHD.
- Learning disabilities. Approximately one-third of patients with TS have a learning disability. Learning disabilities found in TS include difficulties with reading, writing and mathematics, and visual and auditory perception problems. Children with TS can also have dyslexia and problems with retaining information. Some tics seen in TS such as repetitive eye-blinking or head-jerking can make it difficult for the student with TS to read and thus interfere with learning.
- Sleep disorders. Sleep problems such as difficulty falling asleep, waking early, sleepwalking, night terrors and enuresis (bed-wetting) are fairly common in TS. For example, in one study the percentage of different grades of TS patients having trouble getting to sleep ranged from about 45% to 65% as compared to 15% of controls.
- Problems with impulse control. Individuals with TS may display overly aggressive behavior, socially inappropriate acts, self-injurious behavior such as lip biting or banging one's head, and defiant behaviors.
There is no specific lab test or other medical study that can establish the definitive diagnosis of TS. Usually, diagnosis is made through observation of an individual's symptoms and by assessment of family history. Some patients may undergo blood tests, imaging studies such as magnetic resonance imaging (MRI) , or an electroencephalogram (EEG) scan in order to rule out other possible explanations for the symptoms. The process of making a TS diagnosis usually involves monitoring symptoms over a period of several months. The family may be asked to keep records. This period of observation will help determine to what extent the child's symptoms are interfering with ability to function at home, school, and in the community. A neurological examination may be performed. Assessment of cognitive functioning and school performance may be recommended if the child is having difficulty in school.
The American Psychiatric Association published diagnostic criteria, listed below, is from the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition Text Revision (DSM-IV-TR). Another similar set of criteria exists in the International Classification of Disease and Related Health Problems, Tenth Revision (ICD-10). The ICD-10 criteria are not as strict about the age of onset as the DSM-IV-TR criteria.
Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition Text Revision (DSM-IV-TR) criteria
- Both multiple motor and one or more vocal tics present at some time during the illness although not necessarily simultaneously.
- The occurrence of tics multiple times per day (usually in bouts), nearly every day or intermittently during a span of more than one year without a tic-free period of more than three consecutive months.
- The disturbance causes significant distress or impairment in social, occupational, or other important areas of functioning.
- Onset before age 18.
- The disturbance is not due to the direct physiological effects of a substance or a general medical condition.
Treatment of TS disorders requires a multidisciplinary approach. In addition to the patient's primary health care professionals, medical professionals involved in the care of patients with an MPS usually includes specialists in neurology, psychiatry, psychology, social work, genetics, and education. Tourette syndrome support groups may help families in coping with this condition.
There is no cure for TS. Management of TS requires integration of behavioral, psychological and sometimes pharmacologic (medication) therapies. Occupational therapy may also be indicated for TS patients. The decision to treat an individual case of TS depends on the degree to which the symptoms interfere with that person's ability to function. Treatment is crucial in helping the affected child avoid depression , social isolation, and strained family relationships. In general, pharamacologic therapy is reserved for patients with severe symptoms. Education about the condition and reassurance are key components of any treatment program.
Various types of behavior therapy may benefit patients with TS. Using a technique known as habit-reversal training, individuals with severe tics are taught how to substitute one tic for another that is more socially acceptable. Also, since stress can exacerbate tics, individuals with TS may find that relaxation techniques and biofeedback can help alleviate stress reactions and reduce tics. Behavior modification may be necessary for children with poor impulse control.
Psychological counseling may help individuals with TS to cope with the social and emotional problems that occur as a result of their symptoms. Depression and self-esteem problems are common among persons with TS. Counseling is especially important for children with TS as they approach adolescence, a time in which tics tend to get worse. Affected children and their parents may also benefit from family therapy. Severe or frequent tics and the presence of co-occurring problems such as ADHD and OCD can negatively impact quality of life for people with TS, especially if family support is inadequate. Parents may have difficulty accepting a diagnosis and in deciding which how best to handle unwanted behaviors. The goal of family therapy is to educate family members about the disorder and to find ways to handle those symptoms that have a negative impact on family members.
No single or combination (more than one) drug therapy offers complete cessation of symptoms without adverse effects.
Pharmacologic treatment of TS (alone, without OCD or ADHD) usually begins with a trial of clonidine. If clonidine is unsuccessful, treatment moves to one of the dopamine receptor antagonists. Haloperidol, a dopamine receptor antagonist, has been the main drug used in TS treatment since the 1960s. It has been reported that over 80% of patients show improvement of tics with this therapy. A similar drug known as pimozide has also been used as an anti-tic drug since the 1980s. Due to adverse effects associated with haloperidol and pimozide, other dopamine receptor antagonists, including risperi-done, sulpiride (not available in North America as of 2003), and olanzipine have gradually displaced haloperidol and pimozide as the main drug therapies for tics. Newer drugs, ziprasidone and quetiapine, may also be effective; as of 2003, evidence regarding their efficacy was preliminary. For those individuals who are unable to tolerate the above medications, treatment with a related medication, tetrabenazine, may be recommended. Drug therapy with a dopamine agonist may be attempted if none of the above drugs are effective. There are preliminary reports of positive therapeutic effects with other treatments including nicotine, tetrahydrocannbinol (marijuana), baclofen, and botulinum toxin injection yet confirmation of safety and efficacy of these treatments awaits further study.
Clomipramine or one of the selective serotonin uptake inhibitors (SSRIs) are the first choice for treatment of OCD. Examples of SSRI's in use for OCD treatment include fluoxetine, fluvoxamine, sertraline, paroxetine, and citalopram. For those TS patients with OCD who do not respond to SSRIs alone, addition of a dopamine receptor antagonist such as haloperidol, pimozide, risperidine, or olanzipine may be indicated. New therapies under investigation for the treatment of OCD as of 2003 included neurosurgery, deep brain stimulation (DBS), transcranial magnetic stimulation (TMS), and injection with botulinum toxin.
Methylphenidate and dextroamphetamine, medications known as psychostimulants, have been shown to be safe and effective in the treatment of ADHD in TS patients. There has been controversy over the use of psychostimulants to treat ADHD due to concerns about worsening of tics. Results from a randomized, placebo-controlled clinical trial reported in 2002 indicated that methylphenidate and another drug, clonidine, do not adversely affect tics. The researchers also found that a combination of the drugs is more effective than either drug alone.
Recovery and rehabilitation
Children with TS may require academic and occupational interventions. For some TS students, modifying the school environment can help to minimize stress. For the student with vocal tics, untimed exams in a private room and permission to leave the classroom when tics become problematic may help. Children with auditory processing difficulties and fine motor skill problems may benefit from occupational therapy. For example, the use of tape recorders, typewriters, or computers may be recommended to help with reading and writing. Occupational therapy can also help with poor handwriting, a common problem in children with TS. Some students with TS may be eligible for an Individual Education Plan (IEP). An IEP provides a framework from which administrators, teachers, and parents can meet the educational needs of a child with TS. Depending upon severity of TS symptoms and the degree of learning difficulties, some children with TS may be best served by special education classes or a private educational setting.
As of December 2003, thirteen clinical trials were actively recruiting patients with Tourette syndrome. The National Institute of Neurological Disorders and Stroke (NINDS) in Bethesda, Maryland, were sponsoring the following trials. Information on these trials can be found at <http://www.clinicaltrials.gov> or by contacting the Patient Recruitment and Public Liaison Office at 1-800-411-1222 or at email@example.com.
- Magnetic Resonance Spectroscopy (MRS) to Evaluate Tourette's Syndrome. This study will use magnetic resonance imaging (MRI ) and magnetic resonance spectroscopy (MRS) of the brain to try to gain a better understanding of the disease process in Tourette's syndrome. More information can be found at the National Institutes of Health (NIH) web link, <http://clinicalstudies.info.nih.gov/detail/A_2002-N-0128.html>.
- Study of Tics in Patients with Tourette's Syndrome and Chronic Motor Tic Disorder. This study will investigate which areas of the brain are primarily involved in and responsible for tics in patients with Tourette's syndrome and chronic motor tic disorder. More information can be found at the National Institutes of Health (NIH) web link, <http://clinicalstudies.info.nih.gov/detail/A_2002-N-0175.html>.
- Study of GABA-A receptors in the Generation of Tics in Patients with Tourette's Syndrome. This study will investigate how the brain generates tics in patients with Tourette's syndrome and which areas of the brain are primarily affected. More information can be found at the National Institutes of Health (NIH) web link, <http://clinicalstudies.info.nih.gov/detail/A_2002-N-0181.html>
- Brain Dynamics Involved in Generating Tics and Controlling Voluntary Movement. This study will use electroencephalography (EEG) and electromyography (EMG) to examine how the brain generates tics and controls voluntary movement in patients with Tourette's syndrome and chronic motor tic disorder. More information can be found at the National Institutes of Health (NIH) web link, <http://clinicalstudies.info.nih.gov/detail/A_2003-N-0126.html>.
- Brain Activation in Vocal and Motor Tics. This study will investigate the brain areas that are activated by vocal and motor tics in patients with Tourette's syndrome and other tic disorders. More information can be found at the National Institutes of Health (NIH) web link, <http://clinicalstudies.info.nih.gov/detail/A_2002-N-0027.html>.
The following trials were being sponsored by the National Center for Research Resources (NCRR) and coordinated by the Yale University School of Medicine in New Haven, Connecticut. Information on these trials can be found at <http://www.clinicaltrials.gov> or by contacting the study chair, James F. Leckman at 203-785-7971.
- Study of the Neurobiology of Tourette Syndrome and Related Disorders. This study will investigate the patho-biology of Tourette syndrome and related disorders by measuring various compounds of interest in cerebrospinal fluid, plasma, and urine of patients with Tourette syndrome, obsessive compulsive disorder, and/or chronic tics; determine the pattern of familial aggregation of Tourette syndrome and obsessive compulsive disorder by systematic assessment of all first-degree family members of patients selected for cerebrospinal fluid studies; and establish the neurochemical and neuropeptide profile associated with the range of expression of the putative Tourette gene expression in adult and adolescent patients.
- Developmental Phenomenology of Obsessive Compulsive Disorder and Tourette Syndrome in Children and Adolescents. This study will characterize the natural history, associated features, and severity of symptoms of obsessive compulsive disorder and Tourette syndrome in children and adolescents, and identify factors that influence the clinical course and prognosis of these patients.
The National Institute of Mental Health (NIMH) in Bethesda, Maryland, was sponsoring the following trials. Information on these trials can be found at <http://www.clinicaltrials.gov>.
- Brain Tissue Collection for Neuropathological Studies. This study will collect and study the brain tissue of deceased individuals to learn more about the nervous system and mental disorders. More information can be found at the National Institutes of Health (NIH) web page for this study at <http://clinicalstudies.info.nih.gov/detail/A_1990-M-0142.html> or by contacting Joel E. Kleinman, MD at (301) 402-7909 or firstname.lastname@example.org.
- Evaluation and Follow-up of Individuals with Obsessive-Compulsive Disorder and Related Conditions. This study will aim to better understand the long-term progress of people with obsessive-compulsive disorder (OCD) and related conditions such as anorexia nervosa, Tourette syndrome, and trichotillomania. More information can be found at the National Institutes of Health (NIH) web page for this study at <http://clinicalstudies.info.nih.gov/detail/A_2000-M-0067.html> or by contacting the patient recruitment and public liaison office at (800) 411-1222 or email@example.com.
- Brain Imaging of Childhood Onset Psychiatric Disorders, Endocrine Disorders, and Healthy Children. This study will use MRIs to assess brain anatomy and function in normal volunteers and patients with a variety of childhood onset psychiatric disorders. More information can be found at the National Institutes of Health (NIH) web page for this study at <http://clinicalstudies.info.nih.gov/detail/A_1989-M-0006.html> or by contacting the patient recruitment and public liaison office at (800) 411-1222 or firstname.lastname@example.org.
- Treatment of Obsessive-Compulsive Disorder. This study aims to find the best treatment for TS-spectrum obsessive-compulsive disorder (OCD), which includes symptoms of TS, e.g., repeated and involuntary body movements (tics). More information can be found by contacting the University of Florida at email@example.com; the study chair, Wayne Goodman, MD at (877) 788-3994 or firstname.lastname@example.org; or Candy Hill at (352) 392-3681 or email@example.com.
- Genetics of Obsessive-Compulsive Disorder. This study to identify genes that affect susceptibility to obsessive-compulsive disorder (OCD). More information can be found at the National Institutes of Health (NIH) web page for this study at <http://clinicalstudies.info.nih.gov/detail/A_1996-M-0124.html> or by contacting Diane M. Kazuba at (301) 496-8977 or firstname.lastname@example.org. nih.gov.
- Central Mechanisms in Speech Motor Control Studied with H215O PET. This study will use radioactive water (H215O) and Positron Emission Tomography (PET scan) to measure blood flow to different areas of the brain in order to better understand the mechanisms involved in speech motor control, and is sponsored by the National Institute on Deafness and Other Communication Disorders (NIDCD) in Bethesda, Maryland. More information can be found at the National Institutes of Health (NIH) web page for this study at <http://clinical studies.info.nih.gov/detail/A_1992-DC-0178.html> or by contacting the patient recruitment and public liaison office at 1-800-411-1222 or email@example.com.
The majority of cases of TS are mild and as such they do not require medical attention. Most affected individuals show improvement of symptoms in late adolescence or early adulthood and up to one-third of people will experience remission of tic in adult years. In fewer than 10% of patients, tics become more severe in adulthood. TS is not a degenerative disease and patients can anticipate a normal life span.
All students with TS need an educational environment that is supportive and flexible. Children with TS frequently have problems in school because they are teased by peers and misunderstood by teachers. It is important to educate the students, the teachers, and other school personnel who come in contact with the child with TS about the disorder.
American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, 4th edition, text revision (DSM-IV-TR). Washington, DC: American Psychiatric Association, 2000.
Comings, D. E. Search for the Tourette Syndrome and Human Behavior Genes. Duarte, CA: Hope Press, 1996.
Kurlan, R. Handbook of Tourette Syndrome and Related Tic and Behavioral Disorders. Rochester, NY: Marcel Dekker, 2003.
Leckman, J. F. and D. J. Cohen Tourette's Syndrome—Tics, Obsessions, Compulsions: Developmental Psychopathology and Clinical Care, reprint ed. John Wiley and Sons, 2001.
Robertson, M. M., and S. Baron-Cohen. Tourette Syndrome: The Facts, 1st ed. London: Oxford Press, 1998.
Jankovic, J. "Tourette's Syndrome." New England Journal of Medicine 345 (October 2001): 1184–1192.
Leckman, J. F. "Tourette's Syndrome." Lancet 360 (November 2002): 1577–1586.
Miguel, E. C., R. G. Shavitt, Y. A. Ferraro, S. A. Brotto, and J. B. Diniz. "How to Treat OCD in Patients with Tourette Syndrome." Journal of Psychosomatic Research 55 (July 2003): 49–57.
Pauls, D.L. "An Update on the Genetics of Gilles de la Tourette Syndrome." Journal of Psychosomatic Research 55 (July 2003): 7–12.
Robertson, M. M. "Diagnosing Tourette Syndrome: Is It a Common Disorder." Journal of Psychosomatic Research 55 (July 2003): 3–6.
Sandor, P. "Pharmacologic Management of Tics in Patients with TS." Journal of Psychosomatic Research 55 (July 2003): 419–48.
The National Institute of Neurological Disorders and Stroke (NINDS). Tourette Syndrome Information Page. <http://www.ninds.nih.gov/health_and_medical/disorders/tourette.htm>.
Tourette's Disorder Home Page. <http://www.tourettes-disorder.com//home.html>.
National Tourette Syndrome Association, Inc. 42-40 Bell Boulevard, Bayside, New York 11361-2820. (718) 224-2999 or (888) 4-TOURET (486-8738); Fax: (718) 279-9596. firstname.lastname@example.org. <http://www.tsa-usa.org>.
Tourette Syndrome Foundation of Canada. #206, 194 Jarvis Street, Toronto, Ontario M5B 2B7, Canada. (416) 861-8398 or (800) 361-3120; Fax: (416) 861-2472. email@example.com. <http://www.tourette.ca/index.shtml>.
Dawn J. Cardeiro, MS, CGC
"Tourette Syndrome." Gale Encyclopedia of Neurological Disorders. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/science/encyclopedias-almanacs-transcripts-and-maps/tourette-syndrome
"Tourette Syndrome." Gale Encyclopedia of Neurological Disorders. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/science/encyclopedias-almanacs-transcripts-and-maps/tourette-syndrome
Tourette syndrome (TS) is an inherited disorder of the nervous system, characterized by a variable expression of unwanted movements and noises (tics ).
Tourette syndrome is also known as Gilles de la Tourette syndrome, named after Gilles de la Tourette, a French neurologist who first described the syndrome in 1885. Children with Tourette syndrome show symptoms before the age of 18, usually around age seven, and have symptoms that usually last into adulthood. The symptoms of Tourette syndrome are usually motor and/or vocal tics, although in some children other symptoms such as socially inappropriate comments, and socially inappropriate or self-injurious behaviors sometimes occur. Children with Tourette syndrome are more likely to have obsessive-compulsive disorder (OCD), attention deficient disorder (ADD), and attention deficit hyperactivity disorder (ADHD). The symptoms of Tourette syndrome are extremely variable over time, with some symptoms beginning and some ceasing to be a problem as the child grows. Many people with Tourette syndrome experience a decrease in symptoms as they age, and some people see a complete disappearance of their symptoms.
Tourette syndrome is found in all populations and all ethnic groups, but is three to four times more common in males than females. The exact frequency of Tourette syndrome is unknown, but estimates range from 0.05 percent to 2 percent. Estimates vary widely in part because many people with Tourette syndrome have very mild symptoms and may not seek medical attention. It is estimated that there are about 1,000 new cases of Tourette syndrome diagnosed in the United States every year.
Causes and symptoms
The causes of Tourette syndrome are not fully understood. Most studies agree that symptoms of Tourette syndrome involve the chemicals in the brain that help transmit information from one nerve cell in the brain to another. These chemicals are called neurotransmitters. Some studies suggest that the tics in Tourette syndrome are caused by an increased amount of a neurotransmitter called dopamine. Other studies suggest instead that there is a problem with a different neurotransmitter called serotonin. Still others believe the problem involves other chemicals required for normal functioning of the brain.
Most studies suggest that Tourette syndrome is an autosomal dominant disorder with decreased penetrance. An autosomal disorder is one that occurs because of an abnormal gene on a chromosome that is not a sex-linked chromosome. A dominant disorder means that it only takes one abnormal gene in a pair of genes to have the disorder. Parents each pass one copy of each gene to their child. Because in autosomal dominant disorders one gene is abnormal, people with this disorder have about a 50 percent chance of passing the abnormal gene to their offspring. Decreased penetrance means that not all people who inherit the abnormal gene develop symptoms. There is some evidence that females who inherit the Tourette syndrome gene have a lower probability of exhibiting symptoms than males who inherit the gene.
The principal symptoms of Tourette syndrome include simple and complex motor and vocal tics. Simple motor tics are characterized by brief muscle contractions of only one or a small number of muscle groups. An eye twitch is an example of a simple motor tic. Complex motor tics tend to appear more complicated and purposeful than simple tics and involve coordinated contractions of several muscle groups. Some examples of complex motor tics include the act of hitting oneself or jumping.
Vocal tics are actually manifestations of motor tics that involve the muscles required for producing sound. Simple vocal tics include stuttering , stammering, abnormal emphasis of part of a word or phrase, and inarticulate noises such as throat clearing, grunts, and high-pitched sounds. Complex vocal tics typically involve the involuntary expression of words. Perhaps the most striking example of this is coprolalia, the involuntary expression of obscene or socially inappropriate words or phrases, which occurs in fewer than one-third of people with Tourette syndrome. The involuntary echoing of the last word, phrase, sentence, or sound vocalized by oneself (phalilalia) or by another person or sound in the environment (echolalia) are also classified as complex tics.
The type, frequency, and severity of tics exhibited varies tremendously among individuals with Tourette syndrome. Tourette syndrome has a variable age of onset, and tics can start anytime between infancy and age 18. Initial symptoms usually occur before the early teens; the average age of onset for both males and females is approximately seven years. Most individuals with symptoms initially experience simple muscle tics involving the eyes and the head. These symptoms can progress to tics involving the upper torso, neck, arms, hands, and occasionally the legs and feet. Complex motor tics are usually the latest-onset motor tics. Vocal tics usually have a later onset than motor tics.
Not only is there extreme variability in symptoms among individuals with Tourette syndrome, but individuals commonly experience variability in type, frequency, and severity of symptoms over the course of their lifetime. Adolescents with Tourette syndrome often experience unpredictable and more severe than usual symptoms, which may be related to fluctuating hormone levels and decreased compliance in taking medications. Many people who as children have Tourette syndrome experience a decrease in symptoms or a complete end to symptoms in their adult years.
Several factors appear to affect the severity and frequency of tics. Stress appears to increase the frequency and severity of tics, while concentration on another part of the body that is not involved in a tic can result in the temporary alleviation of symptoms. Relaxation following attempts to suppress the occurrence of tics may result in an increased frequency of tics. An increased frequency and severity of tics can also result from exposure to such drugs as steroids, cocaine, amphetamines, and caffeine . Hormonal changes, such as those that occur prior to the menstrual cycle, can also increase the severity of symptoms.
Other associated symptoms
People with Tourette syndrome are more likely to exhibit non-obscene, socially inappropriate behaviors such as expressing insulting or socially unacceptable comments or performing socially unacceptable actions. It is not known whether these symptoms stem from more general dysfunction of impulse control that might be part of Tourette syndrome.
Tourette syndrome appears to also be associated with attention deficit disorder (ADD), a disorder characterized by a short attention span and impulsivity, and in some cases hyperactivity. Researchers have found that 21 to 90 percent of individuals with Tourette syndrome also exhibit symptoms of ADD.
People with Tourette syndrome are also at higher risk for having symptoms of obsessive-compulsive disorder (OCD), a disorder characterized by persistent, intrusive, and senseless thoughts (obsessions) or compulsions to perform repetitive behaviors that interfere with normal functioning. A person with OCD, for example, may be obsessed with germs and may counteract this obsession with continual hand washing. Symptoms of OCD are present in 1.9 to 3 percent of the general population, whereas 28 to 50 percent of people with Tourette syndrome have symptoms of OCD.
Self-injurious behavior (SIB) is also seen more frequently in those with Tourette syndrome. Approximately 34 to 53 percent of individuals with Tourette syndrome exhibit some form of self-injuring behavior. The SIB is often related to OCD but can also occur in those with Tourette syndrome who do not have OCD.
Symptoms of anxiety and depression are also found more commonly in people with Tourette syndrome. It is not clear, however, whether these are symptoms of Tourette syndrome or occur as a result of having to deal with the symptoms of moderate to severe Tourette syndrome.
People with Tourette syndrome may also be at increased risk for having learning disabilities and personality disorders and may be more predisposed to such behaviors as aggression, antisocial behaviors, severe temper outbursts, and inappropriate sexual behavior.
When to call the doctor
Parents should call the doctor if they notice the symptoms of Tourette syndrome. The initial tics usually initially involve the face or head, but the doctor should be consulted if any uncontrolled repetitive behavior is observed.
Tourette syndrome cannot be diagnosed through laboratory tests. Sometimes laboratory tests can be helpful, however, in ruling out other possible conditions. The diagnosis of Tourette syndrome is made by observing and interviewing the child, looking at the family's medical history, and talking to the child's family and sometimes to other caregivers. The diagnosis of Tourette syndrome is complicated by a variety of factors. The extreme range of symptoms of this disorder can make it difficult to differentiate Tourette syndrome from other disorders with similar symptoms. Diagnosis is further complicated by the fact that some tics appear to be within the range of normal behavior. For example, an individual who only exhibits such tics as throat clearing and sniffing may be misdiagnosed with a medical problem such as allergies . In addition, such bizarre and complex tics as coprolalia may be mistaken for psychotic or so-called bad behavior. Diagnosis is also made more difficult because often individuals attempt to control tics in public, and, therefore, the healthcare professional may have difficulty observing the symptoms firsthand. Although there is some disagreement over what criteria should be used to diagnose Tourette syndrome, the most common aid in the diagnosis is the DSM-IV. The DSM-IV outlines suggested diagnostic criteria for a variety of conditions, including Tourette syndrome.
DSM-IV criteria are:
- presence of both motor and vocal tics at some time during the course of the illness
- the occurrence of multiple tics nearly every day through a period of more than one year without a remission of tics for a period of greater than three consecutive months
- distress or impairment in functioning caused by symptoms
- onset occurs prior to age 18
- symptoms not due to medications or drugs and not related to another medical condition
Some physicians criticize the DSM-IV criteria, arguing that they do not include the full range of behaviors and symptoms seen in Tourette syndrome. Others criticize the criteria because they limit the diagnosis to those who experience a significant impairment, which may exclude individuals who have the syndrome but exhibit milder symptoms. For these reasons many physicians use their clinical judgment as well as the DSM-IV criteria as a guide to diagnosing Tourette syndrome.
There is no cure for Tourette syndrome. Treatment involves the control of symptoms through educational and psychological interventions and/or medications. The treatment and management of Tourette syndrome varies from patient to patient and should focus on the alleviation of the symptoms that are most bothersome to the individual or that cause the most interference with daily functioning.
Psychological and educational interventions
Psychological treatments such as counseling are not generally useful for the treatment of tics but can be beneficial in the treatment of associated symptoms such as obsessive-compulsive behavior and attention deficit disorder. Counseling may also help individuals to cope better with the symptoms of Tourette syndrome and to have more positive social interactions. Psychological interventions may also help people cope better with stressors that can normally trigger tics. The education of family members, teachers, and peers about Tourette syndrome can be helpful and may help to foster acceptance and prevent social isolation.
Many people with mild symptoms of Tourette syndrome never require medication. Those with more severe symptoms may require medication for all or part of their lifetime. As of 2004, the most effective treatment of tics associated with Tourette syndrome involved the use of drugs such as haloperidol, pimozide, sulpiride, and tiapride, which decrease the amount of dopamine in the body. Unfortunately, even at low dosages, these drugs bring a high incidence of side effects. The short-term side effects can include sedation, dysphoria, weight gain, movement abnormalities, depression, and poor school performance. Long-term side effects can include phobias , memory difficulties, and personality changes. These drugs are, therefore, better suited for short-term rather than long-term therapy.
In many cases, treatment of associated conditions such as ADD and OCD is considered more important than the tics themselves. Clonidine used in conjunction with such stimulants as Ritalin may be useful for treating people with Tourette syndrome who also have symptoms of ADD. Stimulants should be used with caution in individuals with Tourette syndrome, since they can sometimes increase the frequency and severity of tics. OCD symptoms in those with Tourette syndrome are often treated with such drugs as Prozac, Luvox, Paxil, and Zoloft.
In many cases the treatment of Tourette syndrome with medications can be discontinued after adolescence . Trials should be performed through the gradual tapering off of medications and should always be done under a doctor's supervision.
The prognosis for Tourette syndrome is fairly good. Although symptoms generally get worse during early adolescence, many people with Tourette syndrome experience a lessening of the severity of their symptoms during late adolescence and early adulthood. Approximately one third of children with Tourette syndrome will experience complete or nearly complete remission during their late adolescent and early adult years. Another third will experience a significant drop off in the severity and/or frequency of their symptoms during this time. It is difficult to tell how many children with Tourette syndrome experience complete remission over their entire adult lives, but it has been estimated to be about 8 percent. Many children who do not have complete and lasting remission will experience months or even years without significant symptoms. There does not appear to be a definite correlation between the type, frequency, and severity of symptoms and the eventual prognosis. People with Tourette syndrome who have other symptoms such as obsessive-compulsive disorder, attention deficit disorder, and self-injurious behavior usually have a poorer prognosis.
There is no known way to prevent Tourette syndrome.
Tourette syndrome does not, in itself, negatively affect intelligence or cognition. It is, however, often associated with other disorders such as obsessive-compulsive disorder and attention deficit disorder. It is also sometimes associated with learning and psychological disorders, many of which are often more debilitating than Tourette syndrome itself. Tourette syndrome does not reduce life expectancy. Children with Tourette syndrome often have problems socializing because of embarrassment over uncontrollable tics and negative reactions from parents, teachers, and peers who do not understand the disorder. Children with Tourette syndrome may need special attention to help them cope with the social implications of their disorder.
Attention deficit disorder (ADD) —Disorder characterized by a short attention span, impulsivity, and in some cases hyperactivity.
Autosomal dominant —A pattern of inheritance in which only one of the two copies of an autosomal gene must be abnormal for a genetic condition or disease to occur. An autosomal gene is a gene that is located on one of the autosomes or non-sex chromosomes. A person with an autosomal dominant disorder has a 50% chance of passing it to each of their offspring.
Coprolalia —The involuntary use of obscene language.
Copropraxia —The involuntary display of unacceptable/obscene gestures.
Decreased penetrance —Individuals who inherit a changed disease gene but do not develop symptoms.
Dysphoria —Feelings of anxiety, restlessness, and dissatisfaction.
Echolalia —Involuntary echoing of the last word, phrase, or sentence spoken by someone else.
Echopraxia —The imitation of the movement of another individual.
Neurotransmitter —A chemical messenger that transmits an impulse from one nerve cell to the next.
Obsessive-compulsive disorder —An anxiety disorder marked by the recurrence of intrusive or disturbing thoughts, impulses, images, or ideas (obsessions) accompanied by repeated attempts to supress these thoughts through the performance of certain irrational and ritualistic behaviors or mental acts (compulsions).
Phalilalia —Involuntary echoing by an individual of the last word, phrase, sentence, or sound he/she vocalized.
Tic —A brief and intermittent involuntary movement or sound.
Chowdhury, Uttom. Tics and Tourette Syndrome: A Handbook for Parents and Professionals. New York: Taylor & Francis Inc., 2004.
Cohen, Donald J., et al. Tourette Syndrome. London: Lippincott Williams & Wilkins, 2000.
Lechman, James F. Tourette's Syndrome—Tics, Obsessions, Compulsions: Developmental Psychopathology and Clinical Care. New York: John Wiley & Sons, 2001.
Waltz, Mitzi, et al. Tourette's Syndrome: Finding Answers and Getting Help. Cambridge, MA: O'Reilly Media, 2001.
Prestia, Kelly. "Tourette's Syndrome: Characteristics and Interventions." Intervention in School & Clinic, 39, no. 2 (November 2003): 67.
National Tourette Syndrome Association. 42–40 Bell Blvd., Bayside, NY 11361–2820. Web site: <http://tsa-usa.org/>.
Tish Davidson, A.M.
"Tourette Syndrome." Gale Encyclopedia of Children's Health: Infancy through Adolescence. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/tourette-syndrome-0
"Tourette Syndrome." Gale Encyclopedia of Children's Health: Infancy through Adolescence. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/tourette-syndrome-0
Tourette syndrome (TS) is an inherited disorder of the nervous system, characterized by a variable expression of unwanted movements and noises (tics).
The first references in the literature to what might today be classified as Tourette syndrome largely describe individuals who were wrongly believed to be possessed by the devil. In 1885 Gilles de la Tourette, a French neurologist, provided the first formal description of this syndrome, which he described as an inherited neurological condition characterized by motor and vocal tics.
Although vocal and motor tics are the hallmark of Tourette syndrome, such other symptoms as the expression of socially inappropriate comments or behaviors, obsessive compulsive disorder, attention deficit disorder, self-injuring behavior, depression, and anxiety also appear to be associated with Tourette syndrome. Most research suggests that Tourette syndrome is an autosomal dominant disorder, although a gene responsible for Tourette syndrome has not yet been discovered.
Tourette syndrome is found in all populations and all ethnic groups, but is three to four times more common in males than females and is more common in children than adults. The exact frequency of Tourette syndrome is unknown, but estimates range from 0.05% to 3%.
Causes and symptoms
The cause of Tourette syndrome is unknown, although some studies suggest that the tics in Tourette syndrome are caused by an increased amount of a neurotransmitter called dopamine. A neurotransmitter is a chemical found in the brain that helps to transmit information from one brain cell to another. Other studies suggest that the defect in Tourette syndrome involves another neurotransmitter called serotonin; or involves other chemicals required for normal functioning of the brain.
Most studies suggest that Tourette syndrome is an autosomal dominant disorder with decreased penetrance, although this hypothesis has not been proven and may not be true in all families. An autosomal dominant disorder results from a change in one copy of a pair of genes. Individuals with an autosomal dominant disorder have a 50% chance of passing on the changed gene to their children. Decreased penetrance means that not all people who inherit the changed gene will develop symptoms. There is some evidence that females who inherit the Tourette syndrome gene have a 70% chance of exhibiting symptoms and males have a 99% chance of having symptoms. It has been suggested that other genetic and environmental factors may play a role in the development of symptoms in people who inherit the changed gene, but none have been discovered. Some researchers believe that Tourette syndrome has different causes in different individuals or is caused by changes in more than one gene, although these theories are less substantiated. Further research is needed to establish the cause of Tourette syndrome.
Motor and vocal tics
The principal symptoms of Tourette syndrome include simple and complex motor and vocal tics. Simple motor tics are characterized by brief muscle contractions of one or more limited muscle groups. An eye twitch is an example of a simple motor tic. Complex motor tics tend to appear more complicated and purposeful than simple tics, and involve coordinated contractions of several muscle groups. Some examples of complex motor tics include the act of hitting oneself and jumping. Copropraxia, the involuntary display of unacceptable/obscene gestures; and echopraxia, the imitation of the movement of another individual, are other examples of complex motor tics.
Vocal tics are actually manifestations of motor tics that involve the muscles required for vocalization. Simple vocal tics include stuttering, stammering, abnormal emphasis of part of a word or phrase, and inarticulate noises such as throat clearing, grunts, and high-pitched sounds. Complex vocal tics typically involve the involuntary expression of words. Perhaps the most striking example of this is coprolalia, the involuntary expression of obscene words or phrases, which occurs in fewer than one-third of people with Tourette syndrome. The involuntary echoing of the last word, phrase, sentence or sound vocalized by oneself (phalilalia) or of another person or sound in the environment (echolalia) are also classified as complex tics.
The type, frequency, and severity of tics exhibited varies tremendously between individuals with Tourette syndrome. Tourette syndrome has a variable age of onset and tics can start anytime between infancy and age 18. Initial symptoms usually occur before the early teens; the mean age of onset for both males and females is approximately seven years of age. Most individuals with symptoms initially experience simple muscle tics involving the eyes and the head. These symptoms can progress to tics involving the upper torso, neck, arms, hands, and occasionally the legs and feet. Complex motor tics are usually the latest-onset muscle tics. Vocal tics usually have a later onset then motor tics. In some rare cases, people with Tourette syndrome suddenly present with multiple, severe, or bizarre symptoms.
Not only is there extreme variability in clinical symptoms between individuals with Tourette syndrome, but individuals commonly experience a variability in type, frequency, and severity of symptoms over the course of their lifetime. Adolescents with Tourette syndrome often experience unpredictable and variable symptoms, which may be related to fluctuating hormone levels and decreased compliance in taking medications. Adults often experience a decrease in symptoms or a complete end to symptoms.
A number of factors appear to affect the severity and frequency of tics. Stress appears to increase the frequency and severity of tics, while concentration on another part of the body that is not involved in a tic can result in the temporary alleviation of symptoms. Relaxation, following attempts to suppress the occurrence of tics, may result in an increased frequency of tics. An increased frequency and severity of tics can also result from exposure to such drugs as steroids, cocaine, amphetamines, and caffeine. Hormonal changes such as those that occur prior to the menstrual cycle can also increase the severity of symptoms.
Other associated symptoms
People with Tourette syndrome are more likely to exhibit non-obscene, socially inappropriate behaviors such as expressing insulting or socially unacceptable comments or socially unacceptable actions. It is not known whether these symptoms stem from a more general dysfunction of impulse control that might be part of Tourette syndrome.
Tourette syndrome appears to also be associated with attention deficit disorder (ADD). ADD is a disorder characterized by a short attention span and impulsivity, and in some cases hyperactivity. Researchers have found that 21-90% of individuals with Tourette syndrome also exhibit symptoms of ADD, whereas 2-15% of the general population exhibit symptoms of ADD.
People with Tourette syndrome are also at higher risk for having symptoms of obsessive-compulsive disorder (OCD). OCD is a disorder characterized by persistent, intrusive, and senseless thoughts (obsessions) or compulsions to perform repetitive behaviors that interfere with normal functioning. A person with OCD, for example, may be obsessed with germs and may counteract this obsession with continual hand washing. Symptoms of OCD are present in 1.9-3% of the general population, whereas 28-50% of people with Tourette syndrome have symptoms of OCD.
Self-injurious behavior (SIB) is also seen more frequently in those with Tourette syndrome. Approximately 34-53% of individuals with Tourette syndrome exhibit some form of self-injuring behavior. The SIB is often related to OCD but can also occur in those with Tourette syndrome who do not have OCD.
Symptoms of anxiety and depression are also found more commonly in people with Tourette syndrome. It is not clear, however, whether these symptoms are symptoms of Tourette syndrome or occur as a result of having to deal with the symptoms of moderate to severe Tourette syndrome.
People with Tourette syndrome may also be at increased risk for having learning disabilities and personality disorders ; and may be more predisposed to such behaviors as aggression, antisocial behaviors, severe temper outbursts, and inappropriate sexual behavior. Further controlled studies need to be performed, however, to ascertain whether these behaviors are symptoms of Tourette syndrome.
Tourette syndrome cannot be diagnosed through a blood test. The diagnosis is made through observation and interview of the patient and discussions with other family members. The diagnosis, of Tourette syndrome is complicated by a variety of factors. The extreme range of symptoms of this disorder makes it difficult to differentiate Tourette syndrome from other disorders with similar symptoms. Diagnosis is further complicated by the fact that some tics appear to be within the range of normal behavior. For example an individual who only exhibits such tics as throat clearing and sniffing may be misdiagnosed with a medical problem such as allergies. In addition, such bizarre and complex tics as coprolalia may be mistaken for psychotic or "bad" behavior. Diagnosis is also confounded by individuals who attempt to control tics in public and in front of health care professionals and deny the existence of symptoms. Although there is disagreement over what criteria should be used to diagnosis Tourette syndrome, one aid in the diagnosis is the DSMMD (DSM-IV). The DSM-IV outlines suggested diagnostic criteria for a variety of conditions including Tourette syndrome.
- presence of both motor and vocal tics at some time during the course of the illness
- the occurrence of multiple tics nearly every day through a period of more than one year, without a remission of tics for a period of greater than three consecutive months
- the symptoms cause distress or impairment in functioning
- age of onset of prior to 18 years of age
- the symptoms are not due to medications or drugs and are not related to another medical condition
Some physicians critique the DSM-IV criteria, arguing that they do not include the full range of behaviors and symptoms seen in Tourette syndrome. Others criticize the criteria since they limit the diagnosis to those who experience a significant impairment, which may not be true for individuals with milder symptoms. For this reason many physicians use their clinical judgment as well as the DSM-IV criteria as a guide to diagnosing Tourette syndrome.
There is no cure for Tourette syndrome. Treatment involves the control of symptoms through educational and psychological interventions and/or medications. The treatment and management of Tourette syndrome varies from patient to patient and should focus on the alleviation of the symptoms that are most bothersome to the patient or that cause the most interference with daily functioning.
Psychological and educational interventions
Psychological treatments such as counseling are not generally useful for the treatment of tics but can be beneficial in the treatment of such associated symptoms as obsessive-compulsive behavior and attention deficit disorder. Counseling may also help individuals to cope better with the symptoms of this disorder and to have more positive social interactions. Psychological interventions may also help people cope better with stressors that can normally be triggers for tics and negative behaviors. Relaxation therapies may, however, increase the occurrence of tics. The education of family members, teachers, and peers about Tourette syndrome can be helpful and may help to foster acceptance and prevent social isolation.
Many people with mild symptoms of Tourette syndrome never require medications. Those with severe symptoms may require medications for all or part of their lifetime. The most effective treatment of tics associated with Tourette syndrome involves the use of drugs such as haloperidol, pimozide, sulpiride, and tiapride, which decrease the amount of dopamine in the body. Unfortunately, the incidence of side effects, even at low dosages, is quite high. The short-term side effects can include sedation, dysphoria, weight gain, movement abnormalities, depression, and poor school performance. Long-term side effects can include phobias, memory difficulties, and personality changes. These drugs are therefore better candidates for short-term rather than long-term therapy.
Tourette syndrome can also be treated with such other drugs as clonidine, clonazepam, and risperidone, but the efficacy of these treatments is unknown. In many cases, treatment of such as associated conditions such as ADD and OCD is often more of a concern than the tics themselves. Clonidine used in conjunction with such stimulants as Ritalin may be useful for treating people with Tourette syndrome who also have symptoms of ADD. Stimulants should be used with caution in individuals with Tourette syndrome since they can sometimes increase the frequency and severity of tics. OCD symptoms in those with Tourette syndrome are often treated with such drugs as Prozac, Luvox, Paxil, and Zoloft.
In many cases the treatment of Tourette syndrome with medications can be discontinued after adolescence. Trials should be performed through the gradual tapering off of medications and should always be done under a doctor's supervision.
The prognosis for Tourette syndrome in individuals without associated psychological conditions is often quite good, and only approximately 10% of Tourette syndrome individuals experience severe tic symptoms. Approximately 30% of people with Tourette syndrome will experience a decrease in the frequency and severity of tics, and another 30-40% will experience a complete end of symptoms by late adolescence. The other 30-40% will continue to exhibit moderate to severe symptoms in adulthood. There does not appear to be a definite correlation between the type, frequency, and severity of symptoms and the eventual prognosis. Patients with severe tics may experience social difficulties and may isolate themselves from others in fear of shocking and embarrassing them. People with Tourette syndrome who have such other symptoms as obsessive compulsive disorder, attention deficit disorder, and self-injurious behavior usually have a poorer prognosis.
Attention deficit disorder (ADD)— Disorder characterized by a short attention span, impulsivity, and in some cases hyperactivity.
Autosomal dominant— A pattern of genetic inheritance in which only one abnormal gene is needed to display the trait or disease.
Coprolalia— The involuntary expression of obscene words or phrases.
Copropraxia— The involuntary display of unacceptable/obscene gestures.
Decreased penetrance— Individuals who inherit a changed disease gene but do not develop symptoms.
Dysphoria— Feelings of anxiety, restlessness, and dissatisfaction.
Echolalia— Involuntary echoing of the last word, phrase, or sentence spoken by someone else or sound in the environment.
Echopraxia— The imitation of the movement of another individual.
Neurotransmitter— Chemical in the brain that transmits information from one nerve cell to another.
Obsessive compulsive disorder (OCD)— Disorder characterized by persistent, intrusive, and senseless thoughts (obsessions) or compulsions to perform repetitive behaviors that interfere with normal functioning.
Phalilalia— Involuntary echoing of the last word, phrase, sentence, or sound vocalized by oneself.
Tic— Brief and intermittent involuntary movement or sound.
Leckman, James, and Donald Cohen. Tourette's Syndrome: Tics, Obsessions, Compulsions: Development, Psychopathology and Clinical Care. New York: John Wiley & Sons, 1999.
Robertson, Mary. "Tourette Syndrome, Associated Conditionsand the Complexities of Treatment." Brain 123 (2000): 425-462.
National Institute of Neurological Disorders and Stroke. 31 Center Drive, MSC 2540, Bldg. 31, Room 8806, Bethesda, MD 20814. (301) 496-5751 or (800) 352-9424. 〈http://www.ninds.nih.gov〉.
National Tourette Syndrome Association, Inc. 42-40 Bell Blvd., Bayside, NY 11361-2820. (718) 224-2999. Fax: (718) 279-9596. firstname.lastname@example.org.
Tourette Syndrome Foundation of Canada. 194 Jarvis Street, #206, Toronto, ONT M5B 2B7. Canada (800) 361-3120. email@example.com. 〈http://www.tourette.ca〉.
"About Tourette Syndrome." Tourette Help. 〈http://www.tourettehelp.com/pages/patient/about.html〉.
"Tourette's Disorder." Internet Mental Health. 〈http://www.mentalhealth.com/fr20.html〉.
"Tourette Syndrome." Gale Encyclopedia of Medicine, 3rd ed.. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/tourette-syndrome
"Tourette Syndrome." Gale Encyclopedia of Medicine, 3rd ed.. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/tourette-syndrome
Tourette syndrome (TS) is an inherited disease of the nervous system, first described more than a century ago by a pioneering French neurologist, George Gilles de la Tourette. Before they are 18 years of age, patients with TS develop motor tics; that is, repeated, jerky, purposeless muscle movements in almost any part of the body. Patients also develop vocal tics, which occur in the form of loud grunting or barking noises, or in some cases words or phrases. In most patients, the tics come and go, and are often replaced by different sounds or movements. The tics may become more complex as the patient grows older.
TS is three times more common in men than in women. The motor tics, which usually occur in brief episodes several times a day, may make it very hard for the patient to perform such simple acts as tying shoelaces, not to mention work-related tasks or driving. In addition, TS may have negative effects on the patient's social development. Some patients have an irresistible urge to curse or use offensive racial terms (a condition called coprolalia), although these impulses are not under voluntary control. Other people may not enjoy associating with TS patients. Even if they are accepted socially, TS patients live in fear of offending others and embarrassing themselves. In time, they may close themselves off from former friends and even relatives.
It is important to note, however, that the symptoms of Tourette syndrome are not always dramatic and are often overlooked in people with mild cases of the disorder. A 2001 report published in Pain & Central Nervous System Week, in fact, states that TS is much more common than doctors had thought. A study of 1,596 special-education children in Rochester, NY, found that 8% met the criteria for TS, and 27% had a tic disorder. In Rochester's general population, 3% were found to have Tourette syndrome, and 20% had a tic disorder. The rate of 3% in the general population is 50–75 times higher than the usual estimates given.
The tics of TS are often described as involuntary, meaning that patients cannot stop them. This description is not strictly true, however. A tic is a very strong urge to make a certain motion or sound. It is more like an itch that demands to be scratched. Some patients are able to control their tics for several hours, but once they are allowed expression, they are even stronger and last longer. Tics become worse when the patient is under stress , and usually are much less of a problem during sleep.
Some people with TS have trouble paying attention. They often seem grumpy and may have periods of depression . TS patients may think the same thoughts over and over, a mental tic known as an obsession. It is these features that place TS patients on the border between diseases of the nervous system and psychiatric illness. In fact, before research showed that the brains of TS patients undergo abnormal chemical changes, many doctors were convinced that TS was a mental disorder. It still is not clear whether these behaviors are a direct result of TS itself or a reaction to the stress of having to live with the disease.
Causes & symptoms
Tourette syndrome has been linked to parts of the brain known as the basal ganglia, which regulate movements and are involved in concentration, paying attention, and decision-making. Research has also demonstrated that in TS there is a malfunction in the brain's production or use of important substances called neurotransmitters. Neurotransmitters are chemicals that control the signals that are sent along the nerve cells. The neurotransmitters dopamine and serotonin have been implicated in TS; noradrenaline is thought to be the most important stimulant. Medications that mimic noradrena-line may cause tics in susceptible patients.
TS has a genetic component. If one parent has TS, each child has a 50% chance of getting the abnormal gene. Seven of every 10 girls who inherit the gene, and nearly all boys who inherit it, will develop symptoms of TS. Overall, about one in every 2,500 persons has full-blown TS. Three times as many will have some features, usually chronic motor tics or obsessive thoughts. Patients with TS are more likely to have trouble controlling their impulses, to have dyslexia or other learning problems, and to talk in their sleep or wake frequently. Compulsive behavior, such as constantly washing the hands or repeatedly checking that a door is locked, is a common feature of TS. Compulsions are seen in 30–90% of all TS patients.
Recent research findings suggest that Tourette syndrome may also be related to an autoimmune response. A subset of TS patients have symptoms triggered by infection with Group A beta-hemolytic streptococci. In addition, blood serum antibodies against human basal ganglia have been found in patients with TS.
Motor tics in TS can be classified as simple or complex. Simple tics are sudden brief movements involving a single group of muscles or a few groups that may be repeated several times. Complex tics consist of a repeated pattern of movements that can involve several muscle groups and usually occur in the same order. For instance, a boy with TS may repeatedly move his head from side to side, blink his eyes, open his mouth, and stretch his neck. Vocal tics may be sounds or noises that lack all meaning, or repeated words and phrases that can be understood. Tics tend to get worse and better in cycles, and patients can develop new tics as they grow older. The symptoms of TS may get much better for weeks or months at a time, only to worsen later.
The following examples show why TS can be such a strange and dramatic disorder:
- Simple motor tics. These may include blinking the eyes, pouting the lips, shaking or jerking the head, shrugging the shoulders, and grimacing or making faces. Any part of the body may be tensed up or rapidly jerked, or a patient may suddenly kick. Rapid finger movements are common, as are snapping the jaws and clicking the teeth.
- Complex motor tics. These may include jumping, touching parts of the body or certain objects, smelling things over and over, stamping the feet, and twirling about. Some TS patients throw objects, others arrange things in a certain way. Biting, head-banging, writhing movements, rolling the eyes up or from side to side, and sticking out the tongue may all be seen. A child may write the same letter or word over and over, or may tear apart papers and books. Though they do not intend to be offensive, TS patients may make obscene gestures like "giving the finger," or they may imitate any movements or gestures made by others.
- Simple vocal tics. These include clearing the throat, coughing, snorting, barking, grunting, yelping, and clicking the tongue. Patients may screech or make whistling, hissing, or sucking sounds. They may repeat sounds such as "uh, uh," or "eee."
- Complex vocal tics and patterns. Older children with TS may repeat a phrase such as "Oh boy," "All right," or "What's that?" Or they may repeat everything they or others say a certain number of times. Some patients speak very rapidly or loudly, or in a strange tone or accent. Coprolalia (saying "dirty words" or suggestive or hostile phrases) is probably the best known feature of TS, but fewer than one-third of all patients display this symptom.
Behavioral abnormalities that may be associated with TS include attention deficit hyperactivity disorder (ADHD) and disruptive behaviors, including conduct disorder and oppositional defiant disorder, with aggressive, destructive, antisocial, or negativistic behavior. Academic disorders, learning disorders , and sleep abnormalities (such as sleepwalking and nightmares) are also seen in TS patients.
There are no specific tests for TS. TS is diagnosed by observing the symptoms and asking whether relatives have had a similar condition. To qualify as TS, both motor and vocal tics should be present for at least a year and should begin before age 18 (or, some believe, age 21). Often, the diagnosis is delayed because the patient is misunderstood not only at home and at school, but in the doctor's office as well. It may take some time for the patient to trust the doctor enough not to suppress the strangest or most alarming tics. Blood tests may be done in some cases to rule out other movement disorders. A test of the brain's electrical activity (electroencephalograph or EEG) is often abnormal in TS, but not specific. A thorough medication history is very important in making the diagnosis as well, because stimulant drugs may provoke tics or aggravate the symptoms of TS.
Although there is no cure for TS, many alternative treatments may lessen the severity and frequency of the tics. These include:
- Acupuncture . In one study, acupuncture treatment of 156 children with TS had a 92.3% effective rate.
- Behavioral treatments. Some of these can help TS patients control tics. A large variety of these methods exist, some with proven success.
- Cognitive behavioral therapy . This form of therapy helps the patient to change his or her ingrained response to a particular stimulus. It is somewhat effective in treating the obsessive-compulsive behaviors associated with TS.
- Neurofeedback (electroencephalographic biofeedback ). In neurofeedback, the patient learns to control brain wave patterns; it may be effective in reducing the symptoms of TS. There are, however, no data on this modality as a treatment for TS.
- Psychotherapy. This form of treatment can help the TS patient, and his or her family, cope with depression, poor relationships, and other issues commonly associated with TS.
- Relaxation techniques. Yoga and progressive muscular relaxation are believed to help TS, especially when used in combination with other treatments, because they lower the patient's stress level. One small study found that relaxation therapy (awareness training, deep breathing, behavioral relaxation training, applied relaxation techniques, and biofeedback) reduced the severity of tics, although the difference between the treatment group and control group was not statistically significant.
- Stress reduction training. This training may help relieve the symptoms of TS because stress worsens the tics.
- Other alternative therapies. Homeopathy , hypnosis, guided imagery , and eliminating allergy-provoking foods from the diet have all been reported as helping some TS patients.
Most TS patients do not need to take drugs, as their tics do not seriously interfere with their lives. Drugs that are used to reduce the symptoms of TS include haloperidol (Haldol), pimozide (Orap), clonidine (Catapres), guanfacine (Tenex), and risperidone (Risperdal). One interesting recent finding is that the transdermal nicotine patch, developed to help people quit smoking , improves the control of TS symptoms in children who take haloperidol. Use of the patch allows the haloperidol dosage to be cut in half without loss of effectiveness in symptom control.
Stereotactic treatment, which is high-frequency stimulation of specific regions of the brain, was reported to be successful in significantly reducing tics in a TS patient who had failed to respond to other treatments.
Although there is no cure for TS, many patients improve as they grow older, often to the point where they can manage their lives without drugs. A few patients recover completely after their teenage years. Others learn to live with their condition. There is always a risk, however, that a patient who continues having severe tics will become more antisocial or depressed, or develop severe mood swings and panic attacks.
The only known way to prevent TS as of 2004 is for a couple not to have children when one of them has the condition. Any child of a TS parent has a 50% chance of inheriting the syndrome.
Landau, Elaine. Tourette Syndrome. Danbury, CT: Franklin Watts, 1998.
Leckman, James F., and Donald J. Cohen. Tourette's Syndrome—Tics, Obsessions, Compulsions: Developmental Psychopathology and Clinical Care. New York: John Wiley &Sons, Inc., 1998.
The Merck Manual of Diagnosis and Therapy. Edited by Mark H. Beers, MD, and Robert Berkow, MD. Whitehouse Station, NJ: Merck Research Laboratories, 1999.
"Nicotine Patch Could Help Against Condition's Tics." Health & Medicine Week (October 8, 2001).
"Rate of Disease Much Higher Than Had Been Thought." Pain & Central Nervous System Week (November 19, 2001): 9.
Trifiletti, Rosario. "Antistriatal Antibodies in Tourette Syndrome: Not a Simple Story." Neurology Alert 20 (October 2001): 14.
National Institute of Neurological Disorders and Stroke. National Institutes of Health. P.O. Box 5801, Bethesda, MD 20824. (800) 352-9424. <http://www.ninds.nih.gov>.
Tourette Syndrome Association, Inc. 42-40 Bell Boulevard, Bayside, NY 11361-2820. (718) 224-2999. Fax: (718) 279-9596. firstname.lastname@example.org. <http://www.tsa-usa.org>.
"Tourette Disorder." Internet Mental Health. [cited October 2002]. <http://www.mentalhealth.com/fr00.html>.
Rebecca J. Frey, PhD
"Tourette Syndrome." Gale Encyclopedia of Alternative Medicine. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/tourette-syndrome-2
"Tourette Syndrome." Gale Encyclopedia of Alternative Medicine. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/tourette-syndrome-2
A genetic, neurological disorder characterized by motor and vocal tics and associated behavioral features including obsessions and compulsions and hyperactivity.
Tourette syndrome (TS) affects roughly one in every 2,500 persons. The incidence of the condition is at least three times higher in males than in females. Historically, Tourette syndrome has been a largely misunderstood condition; it has been identified as demonic possession, epilepsy, schizophrenia , and other mental disorders, and was formerly thought to be the result of emotional problems due to faulty childrearing. The condition was first identified as a physiological disorder in 1885 by the French neurologist Gilles de la Tourette. Although the causes of Tourette syndrome are still not fully understood, researchers have made substantial progress in understanding and treating the condition.
Tics—sudden, repetitive, involuntary muscular movements—are the hallmark of Tourette syndrome, appearing in two forms: motor and vocal tics. Motor tics are uncontrollable body movements, such as blinking, grimacing, shrugging, or tossing one's head.Vocal tics, which involve the muscles that produce speech, take the form of uncontrolled speech and involuntary noises, including snorting, hissing, yelping, sniffing, grunting, throat-clearing, and yelling. For a diagnosis of Tourette syndrome to be made, the Diagnostic and Statistical Manual (DSM-IV) of the American Psychiatric Association specifies criteria, including multiple motor tics and at least one vocal tic, occurring numerous times every day or almost daily for a period of over one year, with no tic-free period longer than three months, and onset of symptoms before the age of 18. There are two basic types of tics: simple and complex. Simple tics are isolated movements (such as blinking, kicking, or twitching) that involve only one part of the body. Complex tics are more involved and take the form of recognizable actions, such as poking, hitting, biting, and grooming behaviors (such as smoothing one's hair). They also include imitating the actions of others and making involuntary obscene gestures. Complex vocal tics involve recognizable words (or animal sounds) as opposed to simple noises. These may include the repetition of short phrases, such as "Oh, boy," the repetition of a single word, repetition of the words of others (echolalia ), or involuntary swearing (coprolalia), which is one of the most publicized symptoms of the disorder, although it affects fewer than 10% of people with TS.
Besides tics, there are several types of behavior often associated with Tourette syndrome. At least half the persons affected with TS show symptoms of obsessive-compulsive disorder (OCD), a psychological condition that involves repeated intrusive and senseless thoughts (obsessions) and repetitive behavior (compulsions) intended to stop them. An obsession may be an ordinary but inappropriately intense desire (such as a preoccupation with visiting a certain store) or an outlandish idea, such as a wish to walk across the dinner table or touch a stranger. Compulsions are pointless activities that a person with OCD can not help repeating, such as turning lights on and off, counting things over and over, or arranging objects in a certain pattern. OCD symptoms can be extremely debilitating, taking time away from normal pursuits, including schoolwork and social activities. The other major behavior disorder associated with Tourette syndrome is attention deficit/hyperactivity disorder (ADHD), whose symptoms include hyperactivity, inability to concentrate, and impulse control disorders. Some persons with Tourette syndrome have both OCD and ADHD.
Causes and onset of Tourette syndrome
Tourette syndrome, once thought to be caused by psychological problems, is now known to be a genetic
TOURETTE SYNDROME ASSOCIATION, INC.
The Tourette Syndrome Association (TSA) is a national voluntary non-profit membership organization whose mission it is to identify the cause, find the cure for and control the effects of, Tourette Syndrome (TS). TS is a neurological disorder characterized by various motor and vocal tics, ranging from mild to severe. Members of TSA include those with the disorder, their families, and other interested and concerned individuals.
The Tourette Syndrome Association was founded in 1972 in order to disseminate information to interested individuals, health professionals and agencies in the fields of education and government and to coordinate support groups for affected individuals and their families. TSA also funds research to find the cause of TS and its ultimate cure and to find improved medications and treatments. Today, its membership includes many thousands of individuals and organizations.
The activities of TSA are diverse, ranging from support and counseling to the publication of educational materials. For instance, TSA offers direct help to families in crisis situations through its National Service Response Team. In addition, the Association maintains a data base of those diagnosed with TS, sponsors a Brain Bank Program for collection of tissue needed for research, and maintains a state-by-state list of doctors who diagnose and treat TS. The Association also represents the interests of members to the government on crucial policy issues.
The Tourette Syndrome Association, Inc. has available an extensive list of publications and video tapes concerning symptoms, diagnosis and treatment options for TS discussed in detail. TSA also publishes a quarterly newsletter outlining the latest treatments, research programs and scientific discoveries.
disorder. About 90% of children with TS have a family history of TS or related disorders, such as other conditions involving tics. Some persons are genetic carriers of Tourette syndrome without actually having symptoms themselves (these are almost always females; roughly 99% of males who carry the genetic tendency toward the disorder develop symptoms). The biological basis for Tourette syndrome is an imbalance in the brain's neurotransmitters, chemicals that transport messages between nerve cells. The main neurotransmitter affected in people with TS is dopamine, which controls movement. Research has shown that two other neurotransmitters, norepinephrine and serotonin, also play a role in the condition. In addition, imaging techniques, such as brain scans, have shown abnormalities in the size and functioning of certain parts of the brain in persons affected by TS.
Symptoms of Tourette syndrome usually appear before the age of 18. Children with TS develop their first tics at the age of six or seven, but show other signs of the disorder, including sleep problems, language difficulties, and oppositional behavior, in early childhood , often by the age of two or three. TS usually starts with a single tic, often in the head area (most frequently repeated blinking). The initial tics are generally simple motor tics in the head and upper extremities. As the disorder progresses, the tics gradually move downward to include the torso and lower extremities. Vocal tics usually begin at about the age of nine; complex vocal tics such as coprolalia are among the last to appear. Tics in people with TS are suppressed under certain conditions, usually during sleep and when an individual is engaged in an activity that requires intense concentration. In some cases, children with TS can even manage to keep their tics undercontrol voluntarily in situations where they fear embarrassment, although this takes an immense effort and afterwards the suppressed tics emerge with even greater force than usual. The symptoms of Tourette syndrome increase through childhood and peak during adolescence , after which their intensity usually decreases. An estimated 20-30% of all children with TS outgrow the condition entirely by adulthood.
Although there is no medical cure for Tourette syndrome, medications can relieve many of its symptoms. Currently, the medications of choice for the suppression of tics are antihypertensives, notably Catapres, which reduces tics by 60% in most patients with only minor side effects. Related drugs that have proven effective in tic suppression are Tenex, another antihypertensive, and Klonopin, an antianxiety medication. Another class of drugs, theneuroleptics (including Haldol, Orap, and Prolixin) are even more effective than antihypertensives in suppressing tics, but for most children their advantages are outweighed by side effects, including concentration and memory impairment, weight gain, and drowsiness.
In addition to drugs used for the suppression of tics, additional medications are used to treat other behavioral symptoms associated with Tourette syndrome. Antidepressants , such as Prozac and Anafranil, are effective in treating obsessive compulsive symptoms, and ADHD is commonly treated with Ritalin or other stimulants. Combining these different types of medications can be a difficult balancing act, and their effects need to be carefully monitored by both parents and physicians. For example, the Ritalin used for ADHD may worsen a child's tics, and tricyclic antidepressants such as Norpramin and Anafranil may have to be considered as an alternative treatment for ADHD symptoms. Another symptom of Tourette syndrome that is sometimes treated with medication is uncontrolled aggression , which may be decreased by Tegretol or lithium carbonate. Although medications are universally considered the first line of treatment for Tourette syndrome, relaxation techniques, including self-hypnosis, can also be very helpful in reducing symptoms of the disorder, which worsen with tension. Physical activity is also an excellent way for children with TS to reduce tension and work off their extra energy.
Effects on schoolwork
In spite of the variety of possible symptoms associated with Tourette syndrome, about half of all children who have the disorder require only minor adjustments in order to function successfully in school. The rest require special educational programs to accommodate their needs. TS can disrupt a child's schoolwork in a number of different ways. Tics can make it difficult to concentrate or to perform certain tasks. Ironically, the effort required to suppress them can be just as disruptive because it requires so much energy. Tics can also interfere with the normal school experience by impeding the development of social skills if youngsters feel ostracized by their peers because of their unusual behavior. OCD symptoms also interfere with school performance because preoccupation with obsessive thoughts and the time spent performing compulsive actions make it difficult for children to concentrate on and complete theiracademic tasks. Children whose TS symptoms include ADHD have trouble with the organizational and concentration skills and the self-control needed for successful performance in school. Fortunately, medication helps alleviate tics and symptoms of OCD and ADHD in many children, giving them a better chance of succeeding in school. However, about 40% of children with Tourette syndrome often have additional learning disabilities that require attention, including problems with reading, math, handwriting, and spelling. In many children with TS, educational problems peak between the ages of 11 and 13 and then gradually decrease in severity. Parents of children with Tourette syndrome whose symptoms interfere with their ability to learn in a regular classroom environment should become familiar with their children's rights to an individualized education program under Public Law 94-142, the 1975 federal law aimed at insuring an adequate education for children with special needs.
Baton Rouge Tourette's Support Group. Toughing Out-Tourette's. Baton Rouge, LA: Baton Rouge Tourette's Support Group, 1989.
Buehrens, Adam. Hi, I'm Adam. Duarte, CA:Hope Press, 1991. [Juvenile]
Bruun, Ruth Dowling, and Bertel Bruun. A Mind of Its Own: Tourette's Syndrome, A Story and a Guide. New York: Oxford University Press, 1994.
Comings, David. Tourette Syndrome and Human Behavior. Duarte, CA: Hope Press, 1990.
Fowler, Rick. The Unwelcome Companion: An Insider's View of Tourette Syndrome. Cashiers, NC: Silver Run Publications, 1995.
Haerle, Tracy, ed. Children with Tourette Syndrome: A Parents' Guide. Rockville, MD: Woodbine House, 1992.
Koplewicz, Harold. It's Nobody's Fault: New Hope and Help for Difficult Children and Their Parents. New York: Random House, 1996.
Kurlan, Roger, ed. Handbook of Tourette's Syndrome and Related Tic and Behavioral Disorders. New York: M. Dekker, 1993.
Seligman, Adam, and John S. Hilkevich, eds. Don't Think-About Monkeys: Extraordinary Stories by People with Tourette Syndrome. Duarte, Calif.: Hope Press, 1992.
Tourette Syndrome Association, Inc. 42-40 Bell Boulevard, Bayside, NY 11361–2820, (800) 237–0717, (718) 224–2999.
Tourette Syndrome Clinic. City of Hope National Medical Center. 1500 E. Duarte Rd., Duarte, CA 91010, (818) 359–8111.
"Tourette Syndrome." Gale Encyclopedia of Psychology. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/tourette-syndrome-3
"Tourette Syndrome." Gale Encyclopedia of Psychology. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/tourette-syndrome-3
Tourette syndrome (TS; pronounced ter-ET sin-drohm) is an inherited disorder of the nervous system. It is named after the French physician George Gilles de la Tourette who first described the condition more than a century ago. The condition usually appears before the age of eighteen. Patients develop a pattern of motor tics. A motor (muscular) tic is a repeated, jerky action, such as twisting one's head from side to side. The action has no functional purpose and can occur in any part of the body.
Patients also develop vocal tics. Vocal tics include loud grunts or barking noises. They may also include words or short phrases. In most cases, the tics come and go, and are replaced by different types of sounds and movements. As the patient grows older, the tics may become quite complex.
Motor tics usually occur in bouts that take place several times a day. The tics may make it difficult for a patient to perform simple everyday tasks such as tying one's shoelaces. More complex tasks, such as driving a car, are even more difficult.
Vocal tics can be the source of serious social problems. Some TS patients feel an irresistible urge to shout swear words or offensive racial terms. The patient finds it nearly impossible to control these urges. Some people find it hard to be in the company of a TS patient for this reason. Family members and friends often learn to adjust to these vocal tics. But TS patients usually live in fear of shocking other people or embarrassing themselves. They may withdraw from contact with other people.
Tourette Syndrome: Words to Know
- The process of providing a person with information on some body function, such as blood pressure, so that it can be controlled to some extent.
- A very strong urge to do or say something that usually cannot be resisted for long.
- Difficulty in reading, spelling, and/or writing words.
- Genetic disorder:
- A medical problem caused by one or more defective genes. Genes are passed from one generation to another.
- A muscular contraction or vocal sound over which a patient has very little control.
The tics of TS are often said to be involuntary. That explanation means that a person has no control over the tic. But that description is not exactly correct. The patient is often able to control a tic for several hours at a time. But that control does not last forever. Eventually the tic is allowed to come out. When it does, it is stronger and longer lasting than usual. Tics often become worse when a patient is under stress but are usually less of a problem during sleep.
Some TS symptoms are similar to those of some mental disorders. For example, patients may seem grouchy and depressed. They may tend to think the same thoughts over and over again. This behavior is a kind of mental tic known as obsession. The exact relationship between TS and mental disorders such as obsessive-compulsive disorder (see obsessive-compulsive disorder entry) is still not well understood.
Tourette syndrome is a genetic disorder. That is, the chance of having TS is greatly increased if some other family member also has the condition. If one parent has TS, his or her children have a higher than normal chance of developing the disorder. There is about a 50 percent chance that the child of a TS patient will inherit the TS gene. Male children with the TS gene are three to four times more likely to develop symptoms than are female children with the gene. However, only about 10 percent of those who inherit the TS gene develop symptoms that require medical attention.
Patients with TS can exhibit a range of symptoms. Most patients will have some but not all of the symptoms. Motor tics and obsessive thoughts are the most common symptoms. Compulsive behavior is also common among TS patients. A compulsive behavior is an action performed over and over again for no apparent reason. Constant hand washing is an example of compulsive behavior.
Patients with TS may have other mental disorders. For example, they may have trouble controlling their impulses, or they may talk in their sleep or awaken frequently. Many TS patients also have the learning disorder known as dyslexia (see dyslexia entry).
The tics associated with TS can be simple or complex. A simple tic involves a sudden, brief movement of a single group of muscles. It is generally repeated several times. A complex tic consists of a repeated pattern of movements that can involve several muscle groups, usually in the same order. An example of a complex tic might be a side-to-side head movement followed by eye blinking followed by opening the mouth then stretching the neck.
Vocal tics can consist of sounds and noises that have no meaning. Or they can involve the use of words and phrases repeated over and over again. Tics tend to get better and worse in cycles. Patients often develop new tics as they grow older. The symptoms of TS may get better for weeks or months, only to become worse later.
Some examples of tics associated with TS include the following:
- Simple motor tics: Blinking the eyes, pouting the lips, shaking or jerking the head, shrugging the shoulders, grimacing (making faces), sudden kicking movements, snapping the jaws, and clicking the teeth.
- Complex motor tics: Jumping up and down, touching parts of the body or certain objects, smelling things over and over again, stamping the feet, twirling about, throwing or arranging objects, head-banging, writing the same letter repeatedly, and making obscene gestures.
- Simple vocal tics: Clearing the throat, coughing, snorting, barking, grunting, clicking the tongue, whistling, hissing, and making sucking sounds.
- Complex vocal tics: Repeating phrases such as "Oh boy," "all right," or "what's that?"; saying dirty words or offensive racial expressions; speaking very rapidly or loudly, and repeating whole sentences over and over.
There are no specific tests for TS. However, motor and vocal tics are usually clear symptoms of the condition. A medical history is usually taken to find out if other family members have the condition. The behaviors characteristic of TS must be present for at least a year and should begin before the age of eighteen to justify a diagnosis of Tourette syndrome.
Diagnosis can sometimes be difficult. Many patients learn to hold back their tics when others are around. Family members, school teachers and nurses, and even doctors may not have the chance to observe tics. In some cases, diagnosis is not made until the patient learns to trust a doctor enough to let him or her see the symptoms of TS.
In some cases, tests are conducted to rule out other mental disorders. For example, blood tests may indicate whether or not muscular diseases are present. Electroencephalograms (EEG; pronounced ih-LEK-tro-in-SEH-fuhlo-gram) may show the presence of brain disorders that have symptoms similar to those of TS.
In a majority of cases, patients with TS learn to live with and adapt to their condition. They do not require medication in order to live a reasonably normal life. In serious cases, a drug used to treat severe mental disorders can be used. Haloperidol (pronounced hal-oh-PER-uh-dol, trade name Haldol) or pimozide (pronounced PIM-uh-zide, trade name Orap) are the drugs usually chosen for treatment. They are given at first in low doses and then increased until tics begin to improve.
The drug clonidine (pronounced KLON-uh-deen) is sometimes used with TS patients also. The drug was developed originally to treat high blood pressure. But it may also help relieve the motor tics associated with TS. It usually does not work as well with vocal tics. The drug helps children with TS to focus their attention on tasks. It also makes many patients less frightened of their own symptoms.
Medication is not always the best form of treatment. In many cases, tics often get better by themselves. The best treatment for a patient may a calm, comfortable atmosphere. Children who have TS often do quite well in a regular classroom. If they do require medication, the dose should be monitored carefully. It should be reduced as soon and as much as possible when symptoms begin to get better. Children with severe symptoms may require special classrooms, a private place to study, or individual tutoring.
Counseling will not help cure a person's tics. But it may help the patient understand the nature of his or her condition and how to deal with it. Like any ongoing childhood illness, TS can place great strain on a family. Family counseling can help the TS child, parents, and brothers and sisters better understand the medical condition. Family members can be taught how to be helpful without being over-protective.
Relaxation techniques may reduce the number and severity of tics. Yoga and biofeedback are two such techniques. Homeopathic practitioners believe that patients with TS can be helped with a healthy, well-balanced diet.
There is no cure for Tourette syndrome. However, many patients improve as they grow older and often reach the point where they can do without drugs. A few patients recover completely after their teenage years. Others learn to live with their condition.
TS does not affect one's intelligence or thinking abilities. The most serious difficulty can sometimes be the social problems associated with TS. Patients may become depressed and withdrawn because of embarrassment over their symptoms.
The only way to prevent Tourette syndrome is with family planning. A parent who has TS is likely to pass the condition to his or her own children. Parents may decide to take this information into consideration when deciding whether or not to have children.
FOR MORE INFORMATION
Eisenreich, Jim. Children With Tourette Syndrome: A Parent's Guide. Bethesda, MD: Woodbine House, 1992.
Shimberg, Elaine Fantle, and Oliver Sacks. Living With Tourette Syndrome. New York: Fireside, 1995.
Tourette Syndrome Association. 42-40 Bell Boulevard, Bayside, NY 11361-2820. (718) 224-2999. http://neuro-www2.mgh.harvard.edu/tsa/tsamain.nclk.
Guide to the Diagnosis and Treatment of Tourette Syndrome. Internet Mental Health. [Online] http://www.mentalhealth.com (accessed on October 27, 1999).
"Tourette Syndrome." UXL Complete Health Resource. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/medicine/news-wires-white-papers-and-books/tourette-syndrome
"Tourette Syndrome." UXL Complete Health Resource. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/medicine/news-wires-white-papers-and-books/tourette-syndrome
- * genetically
- means stemming from genes, the material in the body that helps determine a person’s characteristics, such as hair or eye color.
- * central nervous system
- refers to the brain and spinal cord, which coordinates the activities of the entire nervous system. The nervous system is made up of the nerve cells of the body, which communicate with each other to help regulate the functioning of the body.
- * motor
- relates to body movements.
- * tics
- are sudden, brain-activated “involuntary” movements (such as eye blinking or shoulder shrugging) or sounds (words or other sounds, such as sniffing, grunting, throat clearing, or even barking) that are repeated over and over in the same way.
for searching the Internet and other reference sources
Attention deficit hyperactivity disorder
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?
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.
- * chronic
- means lasting for a long time or recurring often.
- * transient
- (TRAN-shent) means brief or producing effects for a short period of time.
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.
- * neurological
- (nur-o-LAH-ji-kal) relates to the nervous system.
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.
- * obsessive-compulsive disorder
- is a condition that causes people to become trapped in a pattern of repeated, unwanted thoughts, called obsessions (ob-SESH-unz), and a pattern of repetitive behaviors, called compulsions (kom-PUL-shunz).
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.
- * dopamine
- (DOPE-a-meen) is a brain chemical that is involved in the control of movement.
- * serotonin
- (ser-o-TOE-nin) is a chemical associated with emotions, particularly feelings of well-being.
- * norepinephrine
- (NOR-e-pi-nefrin) is a body chemical that can increase the arousal response, heart rate, and blood pressure.
- * Parkinson disease
- is a disorder of the central nervous system that causes trembling or rigidity of muscles, uncoordinated movements, and poor balance.
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
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.
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.
- * epilepsy
- is a condition of the central nervous system characterized by recurrent seizures, or violent spasms, that can affect a person’s movements and awareness of the environment.
- * cerebral palsy
- (se-RE-bral PALzee) is a group of conditions, all of which affect a person’s ability to move. They are usually caused by injury to the brain before or soon after birth.
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.
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” (TARdiv 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.
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.
- * degenerative
- means progressively worsening or becoming more impaired.
Attention Deficit Hyperactivity Disorder
Rubio, Gwyn Hyman. Icy Sparks. New York: Viking, 2001. A novel about a 10-year-old girl who tries to conceal her tics and the unhappiness they cause her.
Nemours Center for Children’s Health Media, Alfred I. duPont Hospital for Children, Wilmington, DE. Its website has articles about Tourette syndrome. http://www.KidsHealth.org
National Institute of Neurological Disorders and Stoke, NIH Neurological Institute, P.O. Box 5801, Bethesda, MD 20824. This organization is a leading center for biomedical research and information. Telephone 800–352–9424 http://www.ninds.nih.gov
Tourette Syndrome Association, Inc., 42-40 Bell Boulevard, Bayside, NY 11361-2820. This nonprofit organization for people with TS, family and friends, and health professionals funds research, provides services to patients and their families, and offers various publications and fact sheets. Telephone 718-224-0717 or 800-4-TOURET http://www.tsa-usa.org
"Tourette Syndrome." Complete Human Diseases and Conditions. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/tourette-syndrome-1
"Tourette Syndrome." Complete Human Diseases and Conditions. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/tourette-syndrome-1