Tourette's Disorder

Tourette's disorder, also referred to as Tourette's syndrome, is a neurological disorder characterized by involuntary and uncontrollable tics. A tic is a sudden, rapid, recurrent, nonrhythmic motor movement or vocalization.

Symptoms typically emerge between 4 and 6 years old and can reach their peak between 10 and 12 years old. Around three of every 1,000 people in the United States have Tourette's disorder, and males are affected 2 to 4 times more often than females.

Some people with Tourette's also experience obsessive-compulsive behavior (an intense need to repeatedly perform acts, such as hand washing or checking that a door is locked); attention deficit–hyperactivity disorder (difficulty concentrating and staying on task); learning disabilities (difficulties with reading, writing, and arithmetic); and sleep disorders (frequent awakenings or talking in one's sleep).

Generally, Tourette's disorder is diagnosed by obtaining a description of the tics and evaluating family history. 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 Tourette's. However, Tourette's disorder is a clinical diagnosis. There are no blood tests or other laboratory tests that definitively diagnose the disorder.

Symptoms

  • Multiple motor and one or more vocal tics
  • Tics emerge before age 18
  • Tics have persisted for at least one year
  • Tics cannot be attributed to other conditions such as substance use disorder or Huntington's disease

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, throat clearing, 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, or self-harming behaviors. In 10 to 15 percent of cases, such tics can include saying or shouting inappropriate words and phrases. This symptom of Tourette's disorder is called coprolalia.

People with Tourette's disorder 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 and often improve when the person is relaxed or absorbed in an activity. In most cases, tics decrease markedly during sleep. After delaying tics at school or work, people with Tourette's often seek a secluded spot to release them.

Causes

Although the cause of Tourette's Disorder is unknown, research points to abnormalities in certain brain regions, such as the basal ganglia, frontal lobes, and cortex, the circuits that connect these regions, and the neurotransmitters, such as dopamine, serotonin, and norepinephrine, that are responsible for communication among nerve cells.

As a result of genetic studies in families with tic disorders, risk alleles for Tourette's and rare genetic variants have been identified. It is important for families to understand that genetic predisposition may not necessarily result in Tourette's; it may express itself as a milder tic disorder or as obsessive-compulsive behaviors instead. It is also possible that the gene-carrying offspring will not develop any Tourette's disorder symptoms.

The sex of the person also plays an important role in Tourette's syndrome gene expression. At-risk males are more likely to have tics, and at-risk females are more likely to have obsessive-compulsive symptoms. People with Tourette's may have genetic risks for other neurobehavioral disorders, such as depression or substance abuse. Genetic counseling of individuals with Tourette's should include a full review of all potentially hereditary conditions in the family.

Studies have also shown that obstetrical complications, older paternal age, lower birth weight, and maternal smoking during pregnancy are associated with worse tic severity.

Treatment

Although there is no cure for Tourette's disorder, the condition often improves as the individual matures. Although the disorder is generally lifelong and chronic, it is not a degenerative condition. 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 neurobehavioral disorders such as depression, panic attacks, mood swings, and antisocial behaviors can persist and cause impairment in adult life.

Medication

Because symptoms do not impair most patients, and development usually proceeds normally, the majority of people with Tourette's disorder 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 people with Tourette's, nor does any medication completely eliminate symptoms. Instead, the available Tourette's disorder 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 small doses that are increased slowly until the best possible balance between symptoms and side effects is achieved. Neurological side effects such as tremor, dystonic reactions (twisting movements or postures), Parkinsonian-like symptoms, and other dyskinetic (involuntary) movements are less common and are readily managed with dose reduction. Discontinuing neuroleptics after long-term use must be done slowly to avoid rebound increases in tics and withdrawal dyskinesias. However, side effects can include fatigue, depression, anxiety, weight gain, and difficulties in thinking clearly may be more troublesome.

Clonidine and guanfacine, both antihypertensive drugs, can also treat tics. The most common side effect from these medications that precludes their use is sedation. 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.

Effective medications are also available to treat some of the associated behavioral disorders. Stimulants such as methylphenidate and dextroamphetamine, usually prescribed for attention deficit–hyperactivity disorders, have been reported to be effective without causing tics to become more severe. For obsessive-compulsive behaviors that significantly disrupt daily functioning, fluoxetine, clomipramine, sertraline, or paroxetine may be prescribed.

Therapy

Therapy may also be helpful. Although psychological problems do not cause Tourette's, 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, however. More recently, specific behavioral treatments that include awareness training and competing response training, such as voluntarily moving in response to a premonitory urge, have shown effectiveness in small controlled trials.

Relaxation techniques and biofeedback may be useful in alleviating the stress that can lead to an increase in tic symptoms.

Education

Although students with Tourette's often function well in the classroom, some may have a learning disability. When attention deficit–hyperactivity disorder, obsessive-compulsive disorder, and frequent tics greatly interfere with academic performance or social adjustment, students should be placed in an educational setting that meets their individual needs. These students may require tutoring, smaller or special classes, and in some cases, special schools.

All students with Tourette's disorder need a tolerant and compassionate setting that both encourages them to work to their full potential and provides enough flexibility to accommodate their special needs. This setting may include a private study area, exams outside the regular classroom, untimed testing, or even oral exams when the child's symptoms interfere with his or her ability to write.

References

  • American Psychiatric Association, Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition      
  • American Psychiatric Association, Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition
  • Revised National Institute of Neurological Disorders and Stroke
  • National Institutes of Health National Institutes of Health - National Library of Medicine
  • Tourette Association of America

Last reviewed 02/07/2019