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Tourette's syndrome
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Tourette's syndrome

Explore the available health information, treatment context, and integrative evidence for Tourette's syndrome.

Background
  • Tourette's syndrome (TS), also known as Tourette syndrome or Tourette's disorder, is a neurological disorder characterized by repetitive, involuntary movements and vocalizations called tics. The symptoms are involuntary and cannot be controlled by the individual.
  • The disorder was first described in 1885 in an 86 year-old French noble woman by Dr. Georges Gilles de la Tourette, a pioneering French neurologist.
  • The early symptoms of TS are almost always noticed first in childhood, with the average onset between the ages of seven and ten years. TS occurs in people from all ethnic groups, and males are affected about three to four times more often than females.
  • Overall, about one in every 2,500 persons has TS. Three times as many may exhibit some features of TS, usually chronic motor tics or obsessive thoughts.
  • Individuals with TS have trouble controlling their impulses (they may act out verbally or have involuntary muscle movements), are prone to dyslexia (or other learning problems), and tend to talk during sleep or wake frequently. The nature and complexity of the tics usually change over time with natural variations in frequency (how many times they happen) and severity (the intensity of the symptoms).
  • Obsessive compulsive behavior is common in TS, occurring in 30-90% of all patients. Individuals with obsessions are bothered by thoughts or images that continue to repeat themselves and are almost impossible to ignore. These thoughts, which are annoying, distracting, and inappropriate, tend to cause the person to have moderate to severe anxiety and other emotional discomfort. Common obsessive thoughts include themes of violence, fear of germs and/or infection, and doubts about one's character and/or behavior.
  • Compulsions are behaviors in direct response to the individual's troublesome, obsessive thinking. Therefore, individuals engage in the compulsive behaviors in order to reduce their obsessive thoughts. Some of the most prevalent compulsions are repeated checking of doors, locks, electrical appliances, or light switches; frequent cleaning of hands or clothes; strict attempts to keep various personal items in careful order; and mental activities that are repetitious, such as counting or praying.
  • TS occurs in people from all ethnic groups. It is estimated that 200,000 Americans have the most severe form of TS, and as many as one in 100 exhibit milder and less complex symptoms such as chronic motor or vocal tics or transient tics of childhood.
  • Although there is no cure for TS, symptoms that start in childhood may improve in the late teens and early 20s. As a result, some may actually become symptom-free or no longer need medication for tic suppression. Although the disorder is sometimes lifelong and chronic, it is not a degenerative (worsens over time) condition. Individuals with TS have a normal life expectancy. TS does not impair intelligence.
  • Although tic symptoms may improve with age, disorders such as depression, panic attacks, mood swings, and antisocial behaviors may develop and can persist causing impairment in adult life.
Risk Factors and Causes
  • Genetics: Although no specific gene has been found, evidence from twin and family studies suggests that Tourette's syndrome (TS) is an inherited disorder. Early family studies suggested an autosomal dominant mode of inheritance. An autosomal dominant disorder is one in which only one copy of the defective gene, inherited from one parent, is necessary to produce the disorder. However, more recent studies suggest that the pattern of inheritance is much more complex. If one parent has TS, each child has a 50% chance of getting the abnormal gene.
  • Genetic studies also suggest that some forms of attention deficit/hyperactivity disorder (ADHD) and obsessive compulsive disorder (OCD) are genetically related to TS, but there is less evidence for a genetic relationship between TS and other neurological problems that commonly co-occur with TS, including depression and anxiety. It is important for families to understand 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. It is also possible that the gene-carrying offspring will not develop any TS symptoms.
  • Sex: The sex of the individual plays an important role in TS gene expression. At-risk males are more likely to have tics and at-risk females are more likely to have obsessive-compulsive symptoms. Seven of every ten girls who inherit the gene, and nearly all boys who inherit it, will develop symptoms of TS. The causes are unknown, but hormonal imbalances may be partly responsible.
  • Age: TS usually becomes apparent in children between ages two and 15, with approximately 50% of patients affected by age seven. The age of symptom onset is typically before the age of 18. TS is more frequent in males than females by a ratio of about three or four to one. The disorder is thought to affect 0.1-1.0% of individuals in the general population.
  • Chemical imbalances: Research suggests that the disorder results from abnormalities in the activity of certain chemicals in the brain known as neurotransmitters, particularly dopamine within the basal ganglia. The basal ganglia is the part of the brain responsible for most body functions. In addition, abnormalities in serotonin activity and other neurotransmitters (Brain chemicals), such as norepinephrine, are thought to play some role in causing symptoms associated with TS.
  • Medications: A particularly important risk factor in tics and TS is the use of stimulant medications, such as mixed amphetamine (Adderall®), dextroamphetamine (Dexedrine®), or methylphenidate (Ritalin®). Stimulants are commonly used in individuals with ADHD. Because ADHD is common among children with TS, over 25% of all TS patients have received stimulant therapy. Case reports have found that the use of stimulants is correlated with the onset of motor and phonic tics. Moreover, there is chemical evidence to support the observation that stimulants will increase the severity of tics in 25-50% of TS patients.
  • In many cases, the tics associated with stimulant medication will disappear with the reduction or discontinuation of the medication. There is controversy over whether stimulants can actually trigger or produce prolonged chronic multiple tics or TS that will persist following their termination. However, cases have been reported in which that seems to have occurred. Stimulants may also be drugs of abuse and dependence.
  • Other: Other causes and risk factors for the worsening of TS symptoms include physical health, diet (eating unhealthy foods such as trans fatty acids found in fried foods), and stress level. Non-genetic factors that have been implicated in TS development include stressful processes or events, such as exposure to drugs or other environmental toxins, during the prenatal stage (occurring before birth), perinatal stage (starts at 22 weeks of pregnancy), or up to age two. Environmental toxins include garden chemicals and pesticides or heavy metals like lead and mercury.
Signs and Symptoms
  • The primary characteristics associated with Tourette's syndrome (TS) are multiple motor tics and one or more vocal tics. Motor and vocal tics may develop at about the same time or predominate at different times during the course of the disorder.
  • Early symptoms: The early symptoms of TS are usually noticed first in childhood, with the average onset between the ages of seven and ten years. Mild motor tics and vocal tics may be present.
  • Motor tics: Initially, individuals with TS develop motor tics that are sudden, rapid, recurring, and involuntary muscle movements. Motor tics cause facial grimacing, excessive blinking, and rapid, recurrent movement of the arms, legs, or other areas. Simple motor tics may also include repeated neck stretching, head jerking, or shoulder shrugging. Less commonly, motor tics are more coordinated, having distinct movements involving several muscle groups, such as repetitive squatting, skipping, or hopping. These tics, referred to as complex motor tics, may also include repetitive touching of others, deep knee bending, jumping, smelling of objects, hand gesturing, head shaking, leg kicking, or turning in a circle. In addition to affecting the head and facial area, motor tics also affect other parts of the body, such as the shoulders, torso, arms, and legs. The anatomical locations of motor tics may change over time. Rarely, motor tics evolve to include behaviors that may result in self-injury, such as excessive scratching and lip biting.
  • Vocal tics: Vocal tics are sudden, involuntary, recurrent, often relatively loud vocalizations. Vocal tics usually begin as single, simple sounds that may eventually progress to involve more complex phrases and vocalizations. For example, individuals with TS may initially develop simple vocal tics including grunting, throat clearing, sighing, barking, hissing, sniffing, tongue clicking, or snorting. Complex vocal tics may involve repeating certain phrases or words out of context, one's own words or sounds (palilalia), or the last words or phrases spoken by others (echolalia). Rarely, there may be involuntary, explosive cursing or compulsive utterance of obscene words or phrases (coprolalia).
  • Disease course: Most individuals with TS gradually develop a combination of different motor and vocal tics. These tics may occur a few or many times during the day, often in clusters. Symptoms typically will periodically increase and decrease in frequency and intensity (called waxing and waning). Tics often subside during absorbing activities such as reading or working, decline during sleep, worsen with stress or fatigue, and may be voluntarily suppressed for brief periods. Although TS is considered lifelong, some patients may have weeks or even years with very few or no symptoms. Often tic frequency and severity diminish significantly during adolescence or adulthood. In addition, for many patients, symptoms may completely resolve by early adulthood.
Diagnosis
  • Physical and neurological assessment: The diagnosis of Tourette's syndrome (TS) is mainly based on a thorough clinical evaluation including a complete physical and neurological assessment. A careful patient and family history will also be taken. Neurological assessment may include testing to evaluate cognitive (brain thinking and memory) status, reflexes, balance, and movement, including evaluation of certain voluntary eye movements necessary in focusing on moving targets.
  • The Diagnostic and Statistical Manual of Mental Disorders, 4th ed (DSM-IV-TR) is the standard medical textbook for psychiatric and mental disorders. A diagnosis of TS is based on the following: symptoms of multiple motor tics and one or more vocal tics must be present at the same time, although not necessarily concurrently; the tics must occur many times a day (usually in bouts) nearly every day or intermittently over more than one year, during which time there must not have been a tic-free period of more than three consecutive months; the age at onset must be less than 18 years; and the condition must not be due to the direct physiological effects of a substance (such as stimulants) or a general medical condition (such as Huntington's disease or postviral encephalitis). The doctor will rule out other conditions such as attention deficit/hyperactivity disorder (ADHD) and bipolar disorder.
  • The diagnosis of TS is based upon a thorough clinical evaluation, observation, and assessment of characteristic symptoms and a careful patient and family history. There is no definitive diagnostic test for TS. However, certain blood tests, laboratory studies, or neuroimaging (imaging the nervous system including the brain and spinal cord) techniques may be conducted to eliminate related disorders with similar symptoms.
  • Genetic testing: Since TS has been found to be linked to genes, genetic testing may be used.
  • Neuroimaging techniques: Neuroimaging studies may include computerized tomography (CT) scanning, magnetic resonance imaging (MRI), positron emission tomography (PET) scanning, and electroencephalography (EEG). These tests, however, are rarely needed in most patients with TS.
  • Evaluating children: Although some children are bothered little by tics, other children are greatly affected. To evaluate the effect tics have on a child's life, the doctor will often ask parents questions about areas of the child's life. The child may also need psychological testing and testing for learning problems. It can be difficult to diagnose Tourette's syndrome because tics (or symptoms that look like tics) can be caused by other related disorders such as ADHD. Tests that may be done to check for other conditions include an electroencephalogram (EEG) or computed tomography (CT) scan of the head to see if a person may have seizures or other brain problems. Blood tests may also be done to check for other conditions, including overuse of certain medications (such as amphetamines) or rare medical conditions, such as Wilson's disease (not being able to break down copper in the body).
Complications
  • In addition to tics, there are a variety of behavioral and psychological difficulties that are experienced by many, though not all, patients with Tourette's syndrome (TS). Those behavioral features have placed TS on the border between neurology (nerve study) and psychiatry (dealing with mental illnesses) and require an understanding of both disciplines to comprehend the complex problems faced by many TS patients.
  • The most frequently reported behavioral problems are attention deficits, obsessions, compulsions, impulsivity, irritability, aggression, immaturity, drug and alcohol abuse and addiction, self-injurious behaviors, and depression. Some of the behaviors, such as obsessive compulsive behavior, may be an important part of TS, while others may be more common in TS patients because of certain biological vulnerabilities (such as attention deficit/hyperactivity disorder or ADHD). Still others may represent responses to the social stresses associated with a multiple tic disorder or a combination of biological and psychological reactions.
  • Obsessions and compulsions:
    Although TS may present itself as a disorder of multiple motor and vocal tics, many TS patients also have obsessive-compulsive (OC) symptoms that may be as disruptive to their lives as the tics. There is evidence that obsessive-compulsive symptoms, such as the repetition of tasks, may actually be another expression of the TS gene and, therefore, an integral part of the disorder. Whether this is true or not, it has been well documented that a high percentage of TS patients have OC symptoms, that those symptoms tend to appear somewhat later than the tics, and that they may be seriously impairing.
  • The nature of OC symptoms in TS patients is quite variable. Conventionally, obsessions are defined as thoughts, images, or impulses that intrude on consciousness, are involuntary and distressful, and while perceived as silly or excessive, cannot be abolished. Compulsions consist of the actual behaviors carried out in response to the obsessions or in an effort to ward them off. Typical OC behaviors include rituals of counting, checking things over and over, and washing or cleaning excessively. While many TS patients do have such behaviors, there are other symptoms typical of TS patients that seem to straddle the border between tics and OC symptoms.
  • Attention deficit hyperactivity disorder (ADHD): Up to 50% of all children with TS who come to the attention of a doctor also have attention deficit hyperactivity disorder (ADHD), which is manifested by problems with attention span, concentration, distractibility, impulsivity, and motor hyperactivity. Attention problems often begin before the onset of TS symptoms and may worsen as the tics develop. The increasing difficulty with attention may reflect an underlying biological dysfunction involving inhibition and may be intensified by stresses, such as the strain of working hard to remain quiet and still and suppress involuntary tics. Attention problems and hyperactivity can have a profoundly negative impact on school performance. At least 30-40% of TS children have serious school performance handicaps that require special intervention, and children with both TS and ADHD are especially vulnerable to serious, long term educational impairment. Attention deficits may persist into adulthood. TS together with compulsions and obsessions can lead to significant problems both socially and at work.
  • Emotional changes: Some TS patients have significant problems with mood swings, impulsivity, and aggression directed to others. Temper fits that include screaming, punching holes in walls, threatening others, hitting, biting, and kicking are common, especially in those who also have ADHD. Adults tend to get into fist fights and verbal fights. At times, the temper outbursts result from the internal and external pressures of dealing with TS. A specific cause for such behavioral problems is, however, not well understood. Nevertheless, they create many problems with teachers and great suffering both to TS patients themselves and to their families. Management of those behaviors is often difficult and may involve adjustment of medications, individual therapy, family therapy, or behavioral retraining. The intensity of those behaviors often increases as the tics intensify and decreases as the tics subside.
Treatment
  • There is no known cure for Tourette's syndrome (TS). The goal of therapy is to reduce motor and vocal tics and alleviate associated behavioral problems, such as obsessive-compulsive behaviors, attention deficit/hyperactivity disorder (ADHD), and impulsivity. Behavioral management techniques performed at home, professional counseling, integrative therapies (such as relaxation therapy, biofeedback, and hypnosis), and some medications are effective methods for treating the condition. Brain surgery is currently being studied as a treatment for tics. In mild cases of tic disorder, very little treatment may be needed. Typically, tics increase when the child is tense or stressed, and decrease when the child is relaxed or focused on a task. The use of relaxation techniques or biofeedback may help the child deal with stress. Although some milder tic disorders may respond to behavioral therapies, TS generally does not. The best form of treatment is medication.
  • Medications: A majority of patients with TS do not need to take drugs, as their tics do not interfere much with their lives, and they develop normally.
  • Neuroepilectics: Drug therapy may include low doses of certain antipsychotic medications (called neuroleptics), such as haloperidol (Haldol®), pimozide (Orap®), aripiprazol (Abilify®), fluphenazine (Prolixin®), and risperidone (Risperdal®). These drugs may be effective in reducing the frequency and intensity of tics but should be prescribed with caution, since their use may be associated with certain severe side effects.
  • Side effects associated with neuroleptic therapy include the development of tardive dyskinesia (TD), a movement disorder characterized by persistent, repetitive (stereotypic) involuntary movements usually involving the lower face and mouth.
  • Neuroleptic therapy may also be associated with certain short-term side effects, such as drooling, contraction of the facial and neck muscles, bradykinesia (slow movement), akasthisia (restlessness), and other neurological symptoms.
  • Over the long-term, antipsychotics like risperidone can cause significant weight gain and may lead to diabetes.
  • Although TD sometimes resolves after a period of drug therapy, particularly in children, the condition is not always reversible. Therefore, those who receive long-term neuroleptic therapy should be periodically evaluated to monitor side effects and determine whether dosage level may be decreased or therapy may be discontinued.
  • Botulism injections: Injections of botulinum toxin (BTX or Botox®) into the muscles involved in tics may markedly alleviate not only the abnormal movements but also the premonitory sensations or urges that precede the tics. Pain during injection is usually minimal and subsides rapidly.
  • Other medications: The administration of the anti-anxiety medication clonazepam (Klonopin®) or certain antidepressant medications such as sertraline (Zoloft®), paroxetine (Paxil®), and fluoxetine (Prozac®), may be helpful in the management of some of the associated behavioral symptoms. The antidepressants may also be used for symptoms of obsessive-compulsive disorder (OCD). Side effects include impotence (inability of a man to have or maintain an erection), fatigue (excessive tiredness), weight gain, and drowsiness. Clomipramine (Anafranil®) is also commonly used to control obsessive-compulsive (OC) behavior.
  • Use of clonidine (Catapres®), guanfacine (Tenex®), or alpha 2-adrenergic agonists normally used in hypertension (high blood pressure) may relieve symptoms of ADHD and impulsivity, but these drugs are generally not very effective in controlling tics.
  • Medications may be used in combination when a single agent is only partially effective in reducing the symptoms of TS. Medications used in TS should be tapered gradually to avoid worsening of symptoms when the drug is discontinued. The doctor may simply observe an individual with TS for weeks or even months before starting drug treatment. Usually, after two to three years, TS will be as severe as it is going to get. In the late teenage years, tics often get better by themselves.
  • Psychotherapy: Psychotherapy does not directly impact TS or eliminate tics, but it may be beneficial to some TS patients who develop psychological problems as a result of the condition. The inability to control one's own body and even one's own thoughts can be a great source of anxiety, guilt, fear, helplessness, anger, and depression. Some patients react by withdrawal, others by aggression, and still others by perfectionism and excessive efforts to be in control. Since virtually all TS patients are subjected to some form of negative social reactions, self-esteem problems are common. In addition, TS patients experience all the difficulties associated with growing up with a chronic illness. For those reasons rather (and not for the primary symptoms of TS), counseling may be used.
  • Family treatment: As with any chronic illness, TS can cause great strain on the family as well as on the individual patient. Parents may have a harder time accepting their children's symptoms than the children themselves. Part of the trouble may lie in the guilt associated with the genetic nature of the disorder. Another major problem for parents is distinguishing between the behaviors that are beyond the control of the TS child and the behaviors that can be controlled. Spouses must work together to help solve the complex problems associated with taking care of TS patients. The goal for family members is to develop the flexibility to give special help when needed but not to overprotect.
  • Genetic counseling: With the recognition that TS is familial and genetic, families naturally have become interested in the possibility of genetic counseling. Such counseling must be provided by knowledgeable clinicians who can impart accurate information about the mode of transmission and work with families in dealing with the complex feelings that are aroused.
  • School and work issues: Since TS is an uncommon disorder, schools need to be informed about the nature of TS and the ways it affects attention and learning. Sometimes the doctor will actively serve as a child's advocate. Sometimes children's parents may be asked to keep the child at home if his or her symptoms are thought to be too disruptive for the classroom. This may deprive a child of his or her legal rights for the least restrictive educational environment and adequate education. Parents with children who have TS should seek professional advocacy and support if the child's symptoms are severely disrupting in school.
  • Many adults with TS require special modifications in their working situations. Often, an explanation to the employer about special needs will receive a positive response. Flexibility, compassion, and productivity in the workplace can be increased to everyone's benefit with appropriate interventions for a very symptomatic patient or for a patient who is having difficulty adjusting to a new medication.
  • Surgery: Although still in experimental stages, surgery is being increasingly used for symptomatic treatment of tics in TS. One surgery, called deep brain stimulation (DBS), involves implanting electrodes deep inside the brain, then connecting them under the skin to pacemaker devices on each side of the upper chest. These electrodes use mild electrical stimulation or "shocks" to decrease the neuronal (nerve) firing that causes tics. Some patients report being 85-90% tic-free after the surgery. Risks include infection, headache, and pain. DBS was approved by the U.S. Food and Drug Administration (FDA) in 1997 as a treatment for essential tremor and in April 2003 as a treatment for dystonia (a neurological movement disorder in which sustained muscle contractions cause twisting and repetitive movements or abnormal postures). The FDA approved DBS for Parkinson's disease in 2002.
Prevention
  • Diet: Nutritional changes along with the addition of supplements (vitamins, minerals, and herbs) may be effective in reducing symptoms associated with neurological disorders.
  • It is best to avoid caffeine and other stimulants, alcohol, and smoking.
  • It may be best to eliminate potential food allergens, including dairy (milk, cheese and sour cream), eggs, nuts, shellfish, wheat (gluten), corn, preservatives, and food additives (such as dyes and fillers).
  • Exercise: Maintaining physical fitness is important to those suffering from neurological conditions, such as Tourette's syndrome, as patients who exercise and keep active tend to do better with fewer symptoms and a slower disease progression than those who do not. A daily regimen of exercise can help the person feel better physically and mentally. Individuals should walk as much as possible, even if assistance is necessary. Talking with a healthcare provider about an exercise program is important.
References
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