Tourette Syndrome -Causes, Signs and symptoms

Tourette Syndrome

Also called: TS, Tourette Disease, Gilles de la Tourette Syndrome, Tourette Disorder

Summary

Tourette syndrome (TS) is a neurological disorder characterized by involuntary and repetitive movements and utterances. These may consist of jerking the head, shrugging shoulders, blinking eyes, uttering sounds or explosive vocal outbursts.

Named after George Gilles de la Tourette, the French neurologist who first described the condition, TS may also be referred to as Tourette disease or Tourette disorder. The condition typically becomes apparent in childhood and adolescence. Parents may notice frequently occurring facial tics (e.g., rapid eye blinking, nose twitching or grimacing) in a child. Over time, patients may exhibit more complex motor movements and vocalized tics.

No blood or laboratory tests can diagnose TS. Since some people have minor tics that may come and go, TS is diagnosed only after an individual has experienced motor and vocal tics that recur for at least one year.

Affected individuals report feeling sensations prior to having a tic (called premonitory urges). Some people report the ability to suppress a tic when they feel that one is imminent. However, this suppression does not last indefinitely and the involuntary movement eventually occurs, sometimes more explosively.

An estimated 1 to 2 percent of American children and adults have TS. However, the actual number of people with the condition may be higher because many individuals have minor tics that go undiagnosed. Other risk factors for TS include gender (males are more likely than females to have the condition) and heredity.

There is no cure for TS, which can go into remission or vary in severity or frequency over time. Many children outgrow the condition by as early as age 18, although they may experience periodic recurrences later in life. Minor tics often require no treatment at all. People with more complex TS symptoms may be able to reduce the severity or frequency of their tics by taking medications and employing stress management and coping techniques. People with TS may experience embarrassment and distress over their involuntary movements (especially if they involve socially unacceptable gestures). Children with the condition may become introverted as a result of being ridiculed by their peers. Psychotherapy can help the individual cope with the disorder and reduce stress or anxiety that may trigger tics.

About Tourette syndrome

Tourette syndrome (TS) is a neurological disorder that commonly appears in childhood that is marked by repetitive, involuntary movements and utterances (tics). These tics may be simple (e.g., eye blinking, grunting) or complex (e.g., body twisting, touching objects, repeating words, uttering obscenities).

Named after George Gilles de la Tourette, the French neurologist who first described the condition, TS may also be referred to as Tourette disease or Tourette disorder.

An estimated 200,000 people in the United States have a severe form of TS, according to the National Institute of Neurological Disorders and Stroke (NINDS). Some sources estimate that 1 to 2 percent of American children and adults have the condition. However, the actual number of people with TS may be higher because many individuals may have minor tics that go undiagnosed.

Medical scientists do not completely understand the causes of the disorder. Because TS can manifest in complex motor skill dysfunction, it is likely that a complex mechanism of central nervous system interaction is involved. Environmental factors may also play a role.

Some researchers believe that an abnormality or dysfunction in regions of the brain responsible for movement (e.g., the basal ganglia,cerebral cortex) may contribute to the disorder. The neurons (nerve cells) in these regions send messages via the spinal cord through chemical neurotransmitters such as dopamine, serotonin and norepinephrine. Muscles contract or move in response to signals from these chemicals. However, when these neurons or neurotransmitters do not function properly, communication between the brain and various muscle groups is disrupted, and movement disorders can occur. Medications commonly prescribed to treat TS work by blocking the production of dopamine, serotonin and norepinephrine. This in turn helps to reduce, and in some cases eliminate, the number of tics associated with TS.

Because of the unusual behavior associated with the condition, some people mistakenly believe that individuals with TS have a mental illness.

Many people with TS also tend to have certain other disorders, such as attention-deficit hyperactivity disorder and obsessive-compulsive disorder. There also appears to be an increased risk of depression and substance abuse among patients with TS.

Classification of Tourette syndrome

The involuntary movements and utterances (tics) associated with Tourette syndrome (TS) may be classified as simple or complex. These movements and vocal tics can occur independently or in combination with each other. For example, a person with TS may twitch his nose while grunting or clearing his throat. The motor tic may occur first, followed by the verbal outburst or vice versa.

Simple tics, such as blinking the eyes, sniffing or clearing the throat, usually involve a limited number of muscle groups. Complex tics involve the coordination of multiple muscle groups. Examples of complex tics include hand gestures or repetition of words or phrases.

Examples of motor or movement tics include:

SimpleComplex
Eye blinkingTouching oneself and others
Jerking the head or shoulderKicking, jumping and body gyrations
Nose twitchingPounding the chest, stomach or head with fists
Grimacing or shrugging the shouldersGrimacing accompanied by head twist and shoulder shrug
Teeth grindingAbnormal walking (gait)
Pursing the lipsRepeating the movements of others (echopraxia)

Verbal tics may include:

SimpleComplex
GruntingUse of obscene words (coprolalia)
HiccupsEchoing the words of others (echolalia)
Sniffing, snortingRepetitive speech
Barking 
Clearing the throat 

Risk factors and causes of Tourette syndrome

Medical scientists are not sure exactly what causes Tourette syndrome (TS). Theories about possible causes include:

  • Heredity. Genetic research indicates that TS may be inherited.

  • Brain structure abnormalities. Images of the brains of people with TS show abnormalities in the area of the basal ganglia and cerebral cortex.

  • Brain chemical abnormalities. Dopamine, serotonin and norepinephrine (neurotransmitters in the brain) may play a role in the movement disorders.

  • Infections. Some experts believe there may be a link been childhood streptococcal infections and TS, but there is little or no evidence to support this.

Risk factors for TS include:

  • Family history of the disorder or of other tic or movement disorders.

  • Gender. Males are three to four times more likely to have TS than females.

Signs and symptoms of Tourette syndrome

Children with Tourette syndrome (TS) may begin to show signs or symptoms of the disorder as early as age 2, although signs typically appear between the ages of 7 and 11. Signs of TS typically first appear in the head and neck before occurring in other areas of the body (e.g., trunk, arms, legs). In general, motor tics occur before vocal tics. Simple tics occur prior to the development of more complex tics. The condition is often missed by parents who mistakenly think children may be sniffing or clearing their throat because of allergies or blinking due to vision problems.

In some cases, TS tics are preceded by a strong urge to make a specific motor or vocal movement (premonitory urge). Some patients with TS have described that, in order to relieve this urge, the tic must be performed in a certain way or certain number of times.

A person with TS may exhibit repetitive, involuntary movements or utterances (tics) that may include the following:

  • Facial tics (eye blinking, darting or winking; sticking out the tongue)
  • Head jerking
  • Turning the head
  • Teeth grinding
  • Shoulder flexing or shrugging
  • Finger flexing
  • Stomping the feet
  • Repetitive hand or body gestures
  • Repeating the gestures of others
  • Touching the genitals
  • Verbal tics (hiccups, grunts, yelling, throat clearing, barking, obscene language,  repeating words or phrases and using different voice tones)

These symptoms may range from mild to severe. Their relative intensities can wax and wane over time, although the symptoms may last a lifetime. In most cases, symptoms of TS tend to peak in the mid-teen years and improve during the late teen years and into adulthood. Symptom location and frequency can also vary over time.

Symptoms can be exacerbated by stress, excitement, fatigue or anxiety. Symptoms may improve when a patient is calm and focused. Symptoms may be present during sleep, but are usually significantly diminished. An isolated occurrence of these symptoms does not mean a child has TS. Diagnosis is made after a person has experienced repeated or periodic symptoms of both motor and vocal tics for at least a year.

Diagnosis methods for Tourette syndrome

There are no blood or other laboratory tests to confirm whether an individual has Tourette syndrome (TS). A pediatrician, neurologist or other physician will obtain a complete family medical history and conduct a physical examination of the patient.

Since minor muscle tics are not unusual in some children, parents may be asked to observe and report their child’s symptoms for at least one year. They will also likely be asked the following:

  • Is the tic motor or vocal? Does it occur alone or in combination with other motor or vocal tics?
  • How many times a day or week does the tic occur? Do tics continue during sleep?
  • Where on the body are the tics located? What is the duration of the tic?
  • Has a parent or other observer witnessed the tics or recorded (videotaped) them in any way?

If motor and vocal tics continue beyond a year, the physician may then diagnose TS as a possible cause.

Another important factor in diagnosis is whether the patient experiences urges or feelings prior to having a tic (premonitory urges) and whether they are able suppress the tic. The ability to suppress an involuntary tic distinguishes TS from other movement disorders that are completely uncontrollable.

Physicians may also look for the presence of certain other behavioral disorders that are often present concurrently in individuals with TS. These include attention deficit hyperactivity disorder (ADHD) and obsessive compulsive disorder (OCD).

Atypical symptoms such as adult onset TS may take longer to diagnose. Diagnosis may also be delayed in children who exhibit symptoms only sporadically or whose family members or physicians may overlook the symptoms. Repeated sniffing, for example, may be confused with having allergies and repetitive eye blinking may be mistaken for vision problems.

The neurologist or pediatrician may order one of the following neuroimaging studies (primarily to rule out the presence of other disorders that may be causing the symptoms):

  • Magnetic resonance imaging (MRI). MRI is a noninvasive procedure that uses powerful magnets and radio waves to produce clear, cross-sectional or three-dimensional images of bodily tissues.
  • Computed axial tomography (CAT) scan. A CAT scan is a noninvasive or minimally invasive test that uses a rotating x-ray device and computer to create three-dimensional, cross-sectional images (or slices) of the brain and skull.
  • Electroencephalogram (EEG). During this painless test, small metal devices called electrodes are attached to the scalp. The electrodes are connected by wires (leads) to an electroencephalograph machine that charts the electrical activity of the brain.

Treatment/prevention of Tourette syndrome

There is no cure for Tourette syndrome (TS), which can go into remission or vary in severity or frequency over time. Many children outgrow the condition by as early as age 18, although they may experience periodic recurrences later in life.

For the majority of people with TS, motor and vocal tics are not severe enough to warrant any kind of treatment. When tics are severe or cause behavior that disrupts normal social activities (work, school or daily life), treatment is considered to control the involuntary movements or outbursts.

Severe cases of TS may respond well to medication therapy. Antipsychotic drugs that block dopamine receptors in the central nervous system may help reduce the frequency and severity of tics. In addition, antidepressants (selective serotonin reuptake inhibitors), stimulants and alpha adrenergic inhibitors may also be prescribed to help control tics.

Antidyskinetics may also be recommended to treat TS. These medications are typically prescribed at low doses and slowly increased over time to a level that best controls tics while minimizing side effects. Use of these medications must be weighed against any associated side effects, including sedation.

Patients must be sure to inform their physicians of all prescription and over-the-counter medications or herbal supplements they are taking. In addition, there is a risk of a patient developing tardive dyskinesia (which also involves repetitive, involuntary movement) as a result of using certain medications. Patients’ medications for TS should be monitored over time to ensure that they are always receiving the proper dosage level and to determine whether therapy may be discontinued.

Other treatment options may include:

  • Psychotherapy. Counseling from a mental health professional can help reduce stress and anxiety that often precede TS tics. It may also be helpful in treating any accompanying behavior disorders, such as attention deficit hyperactivity disorder (ADHD) or obsessive compulsive disorder (OCD). Counseling may also help a person cope with the disorder, as well as the social and emotional problems that sometimes result from having TS.
  • Muscular injections. Injections of botulinum toxin (Botox) appear to significantly reduce tics as well as the premonitory urges that precede them.
  • Deep brain stimulation. In rare cases where patients have disabling tics that do not respond to other treatment methods, the neurologist may recommend deep brain stimulation of the globus pallidus, thalamus or other subcortical regions of the brain. The procedure involves using a battery-powered device (neurostimulator) to help control the tics.
  • Complementary and alternative medicine. People have tried using relaxation techniques, biofeedback, cognitive behavior modification, hypnosis and various herbal supplements to reduce the number and severity of tics they experience. However, none of these treatments have been scientifically proven to be effective.

There is no known prevention method for TS. Some people with the condition report the ability to temporarily suppress muscle or vocal tics when they feel premonitory urges. However, the tic eventually occurs, sometimes in a more explosive release.

Lifestyle concerns with Tourette syndrome

People with Tourette syndrome (TS) can live normal, healthy lives. However, depending on its severity, the disorder can negatively affect self-image and self-esteem, personal relationships and school and work achievement. Commonplace activities like dating, vacationing, shopping or going to the movies can be difficult for individuals affected with TS. Some people with TS may become introverts to avoid public exposure of their disorder. Others are at increased risk of depression and alcohol or substance abuse.

Individuals with TS can experience embarrassment and distress over their involuntary movements (especially if they involve socially unacceptable gestures). Children with the condition may be ridiculed by their peers and become introverted as a result.

Experts recommend that people with TS employ the following techniques to help them cope with the disorder:

  • Develop stress management and anxiety reduction techniques
  • Seek help from a mental health professional
  • Attempt to remain occupied and calm (e.g., through physical activity, hobbies, crossword puzzles, games) to help reduce the frequency of tics

Parents may also wish to discuss alternative educational options (e.g., tutoring, smaller classes, home schooling) for school-aged children with TS.

Questions for your doctor regarding TS

Preparing questions in advance can help patients to have more meaningful discussions with healthcare professionals regarding their condition. Patients may wish to ask their doctors the following questions related to Tourette syndrome (TS):

  1. What caused my child’s TS? Is it hereditary?
  2. How can we be sure that the tics are not caused by another disorder?
  3. What medications do you recommend my child take to reduce the number of tics experienced daily?
  4. What are the side effects of the medications prescribed to treat TS?
  5. What other things can I do to help prevent or reduce the number of tics experienced by my child?
  6. What are the chances my child’s tics will completely disappear over time?
  7. Are there any alternative and complementary treatments you can recommend for TS?
  8. How helpful would psychotherapy be in reducing my child’s tics and/or helping them cope with the disorder?
  9. What special classroom or educational arrangements should I discuss with my child’s teachers or school?
  10. How can I find a local support group of parents of children with TS to help me cope?
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