What is Tourette syndrome?

Tourette syndrome (TS) is an inherited, neurological disorder characterized by multiple involuntary movements and uncontrollable vocalizations called tics that come and go over years. In a few cases, such tics can include inappropriate words and phrases. The disorder is named for Dr. Georges Gilles de la Tourette, the pioneering French neurologist who first described an 86-year-old French noblewoman with the condition in 1885. The symptoms of TS generally appear before the individual is 18 years old. TS can affect people of all ethnic groups; males are affected 3 to 4 times more often than females. It is estimated that 100,000 Americans have full-blown TS, and that perhaps as many as 1 in 200 show a partial expression of the disorder, such as chronic multiple tics or transient childhood tics. The natural course of TS varies from patient to patient. Although TS symptoms range from very mild to quite severe, the majority of cases fall in the mild category. What are the symptoms? The first symptoms of TS are usually facial tics--commonly eye blinking. However, facial tics can also include nose twitching or grimaces. With time, other motor tics may appear such as head jerking, neck stretching, foot stamping, or body twisting and bending. TS patients may utter strange and unacceptable sounds, words, or phrases. It is not uncommon for a person with TS to continuously clear his or her throat, cough, sniff, grunt, yelp, bark, or shout. People with TS may involuntarily shout obscenities (coprolalia ) or constantly repeat the words of other people (echolalia). They may touch other people excessively or repeat actions obsessively and unnecessarily. A few patients with severe TS demonstrate self-harming behaviors such as lip and cheek biting and head banging against hard objects. However, these behaviors are extremely rare. Tics alternately increase and decrease in severity, and periodically change in number, frequency, type, and location. Symptoms may subside for weeks or months at a time and later recur. How are tics classified? There are two categories of tics: simple and complex. Simple tics are sudden, brief movements that involve a limited number of muscle groups. They occur in a single or isolated fashion and are often repetitive. Some of the more common examples of simple tics include eye blinking, shoulder shrugging, facial grimacing, head jerking, yelping, and sniffing. Complex tics are distinct, coordinated patterns of successive movements involving several muscle groups. Complex tics might include jumping, smelling objects, touching the nose, touching other people, coprolalia, echolalia, or self-harming behaviors. Can people with TS control their tics? People with TS can sometimes suppress their tics for a short time, but the effort is similar to that of holding back a sneeze. Eventually tension mounts to the point where the tic escapes. Tics worsen in stressful situations; however they improve when the person is relaxed or absorbed in an activity. In most cases tics decrease markedly during sleep. What causes TS? Although the basic cause of TS is unknown, current research suggests that there is an abnormality in the gene(s) affecting the brain's metabolism of neurotransmitters such as dopamine, serotonin, and norepinephrine. Neurotransmitters are chemicals in the brain that carry signals from one nerve cell to another. What disorders are associated with TS? Not all people with TS have disorders other than tics. However, many people experience additional problems such as obsessive compulsive behavior, characterized by an intense need to act repeatedly, such as hand washing or checking that a door is locked; attention deficit-hyperactivity disorder, characterized by difficulty concentrating and staying on task; learning disabilities, which include reading, writing, and arithmetic difficulties; or sleep disorders, which include frequent awakenings or talking in one's sleep. The wide range of behavioral symptoms that can accompany tics may, in fact, be more disabling than the tics themselves. Patients, families, and physicians need to determine which set of symptoms is most disabling so that appropriate medications and therapies can be selected. How is TS diagnosed? Generally, TS is diagnosed by obtaining a description of the tics and evaluating family history. For a diagnosis of TS to be made, both motor and phonic tics must be present for at least 1 year. Neuroimaging studies, such as magnetic resonance imaging (MRI), computerized tomography (CT), and electroencephalogram (EEG) scans, or certain blood tests may be used to rule out other conditions that might be confused with TS. However, TS is a clinical diagnosis. There are no blood tests or other laboratory tests that definitively diagnose the disorder. Studies show that correct diagnosis of TS is frequently delayed after the start of symptoms because many physicians may not be familiar with the disorder. The behavioral symptoms and tics are easily misinterpreted, often causing children with TS to be misunderstood at school, at home, and even in the doctor's office. Parents, relatives, and peers who are unfamiliar with the disorder may incorrectly attribute the tics and other symptoms to psychological problems, thereby increasing the social isolation of those with the disorder. And because tics can wax and wane in severity and can also be suppressed, they are often absent during doctor visits, which further complicates making a diagnosis. In many cases, parents, relatives, friends, or even the patients themselves become aware of the disorder based on information they have heard or read in the popular media. How is TS treated? Because symptoms do not impair most patients and development usually proceeds normally, the majority of people with TS require no medication. However, medications are available to help when symptoms interfere with functioning. Unfortunately, there is no one medication that is helpful to all persons with TS, nor does any medication completely eliminate symptoms; in addition, all medications have side effects. Instead, the available TS medications are only able to help reduce specific symptoms. Some patients who require medication to reduce the frequency and intensity of the tic symptoms may be treated with neuroleptic drugs such as haloperidol and pimozide . These medications are usually given in very small doses that are increased slowly until the best possible balance between symptoms and side effects is achieved. Recently scientists have discovered that long-term use of neuroleptic drugs may cause an involuntary movement disorder called tardive dyskinesia . However, this condition usually disappears when medication is discontinued. Short-term side effects of haloperidol and pimozide include muscular rigidity, drooling, tremor, lack of facial expression, slow movement, and restlessness. These side effects can be reduced by drugs commonly used to treat Parkinson's disease. Other side effects such as fatigue, depression, anxiety, weight gain, and difficulties in thinking clearly may be more troublesome. Clonidine , an antihypertensive drug, is also used in the treatment of tics. Studies show that it is more effective in reducing motor tics than reducing vocal tics. Fatigue, dry mouth, irritability, dizziness, headache, and insomnia are common side effects associated with clonidine use. Fluphenazine and clonazepam may also be prescribed to help control tic symptoms. Medications are also available to treat some of he associated behavioral disorders. Stimulants such as methyphenidate, pemoline , and dextroamphetamine , usually prescribed for attention deficit-hyperactivity disorders, although somewhat effective, have also been reported to increase tics; therefore their use is controversial. For obsessive compulsive behaviors that significantly disrupt daily functioning, fluoxetine , clomipramine , sertraline , and paroxetine may be prescribed. Other types of therapy may also be helpful. Although psychological problems do not cause TS, psychotherapy may help the person better cope with the disorder and deal with the secondary social and emotional problems that sometimes occur. Psychotherapy does not help suppress the patient's tics. Relaxation techniques and biofeedback may be useful in alleviating stress which can lead to an icrease in tic symptoms. Is TS inherited? Evidence from genetic studies suggests that TS is inherited in a dominant mode and the gene(s) involved can cause a variable range of symptoms in different family members. A person with TS has about a 50-50 chance of passing on the gene(s) to one of his or her offspring. However, that genetic predisposition may not necessarily result in full-blown TS; instead, it may express itself as a milder tic disorder or as obsessive compulsive behaviors or possibly attention deficit-hyperactivity disorder with few or no tics at all. It is also possible that the gene-carrying offspring will not develop any TS symptoms. A higher than normal incidence of milder tic disorders and obsessive compulsive behaviors has been found in families of individuals with TS. Gender also plays an important role in TS gene expression. If the gene-carrying offspring of a TS patient is male, then the risk of developing symptoms is 3 to 4 times higher. However, most people who inherit the gene(s) will not develop symptoms severe enough to warrant medical attention. In some cases of TS, inheritance cannot be determined. These cases are called sporadic and their cause is unknown. What is the prognosis? There is no cure for TS; however, the condition in many individuals improves as they mature. Individuals with TS can expect to live a normal life span. Although the disorder is generally lifelong and chronic, it is not a degenerative condition. TS does not impair intelligence. Tics tend to decrease with age, enabling some patients to discontinue using medication. In a few cases, complete remission occurs after adolescence. Although tic symptoms tend to decrease with age, it is possible that neuropsychiatric disorders such as depression, panic attacks, mood swings, and antisocial behaviors may increase. For more information on neurological disorders or research programs funded by the National Institute of Neurological Disorders and Stroke, contact the Institute's Brain Resources and Information Network (BRAIN) at: BRAIN P.O. Box 5801 Bethesda , MD 20824 (800) 352-9424 http://www.ninds.nih.gov Information also is available from the following organizations: Tourette Syndrome Association 42-40 Bell Boulevard Suite 205 Bayside, NY 11361-2820 ts@tsa-usa.org http://tsa-usa.org Tel: 718-224-2999 888-4-TOURET ( 486-8738 ) Fax: 718-279-9596 National Institute of Mental Health (NIMH) National Institutes of Health, DHHS 6001 Executive Blvd. Rm. 8184, MSC 9663 Bethesda , MD 20892-9663 nimhinfo@nih.gov http://www.nimh.nih.gov Tel: 301-443-4513/301-443-8431 (TTY) 866-615-NIMH (-6464) Fax: 301-443-4279 National Institute on Deafness and Other Communication Disorders (NIDCD) National Institutes of Health, DHHS 31 Center Drive , Rm. 3C35 MSC 2320 Bethesda , MD 20892-2320 nidcdinfo@nidcd.nih.govhttp://www.nidcd.nih.gov Tel: 301-496-7243 301-241-1055 (TTD/TTY