Definition

Tourette syndrome (TS) is a chronic, neurological disorder. It is characterized by motor and vocal tics. Both motor and vocal tics must be present, though not necessarily at the same time. Tics must be present for more than one year, and its onset must be prior to age 18. Tics are rapid, involuntary movements or sounds that occur repeatedly.

Causes

In most cases, TS is inherited through a gene (or genes). However, some expressions of the gene(s) may be milder tic disorders or obsessive-compulsive symptoms with no tics. Sometimes there are no symptoms.

Genetic Material

Chromosome_DNA

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Some people with TS have no known family history of TS, tics, or obsessive-compulsive symptoms. Researchers are studying whether other factors, such as birth-related issues and autoimmune problems, may contribute to TS as well.

One of the most complicated questions involves a condition known as “pediatric autoimmune psychiatric disorders” or PANDAS. TS is one of the conditions grouped in PANDAS, and there is much evidence to suggest that, in at least some children, PANDAS (including TS) is associated with Group A streptococcal or “strep” infection of the throat. We do not know yet whether rapid treatment of strep infections or prevention of strep recurrence would reduce the risk of PANDAS. We also do not know yet whether all children are equally at risk of getting PANDAS or whether only children with certain genetic make-up are at risk. In any case strep infections are very common, and if PANDAS is sometimes caused by strep it is likely a very rare consequence.

Other than this possible relationship with streptococcal infection in some children, the cause of the tics has not yet been determined. However, studies show that several brain chemicals, called neurotransmitters, are involved. Dopamine and serotonin are the most likely. In addition, stress and tension often increase tics.

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition. Your risk of TS is increases if:

  • You have a family history of TS, other tic disorders, or obsessive-compulsive disorder
  • You are male (men are affected 3 to 4 times more commonly than women)
  • Strep infection may be a risk in some children

There are many secondary causes of TICS including:

  • Hereditary disorders
  • Carbon monoxide poisoning
  • Traumatic brain injury
  • Cerebral infections
  • Medications
  • Illicit drugs

Symptoms

Symptoms range from mild to severe, but most cases are mild. They are sudden in onset, and have variable duration.

Tics may decrease with concentration and distraction temporarily, and increase in times of stress.

Tics are divided into motor and vocal tics and then subdivided into simple and complex tics. The following are common examples of tics:

Motor Tics:

Simple—eye blinking, facial grimacing, head jerking, arm or leg thrusting

Complex—jumping, smelling, touching things or other people, twirling around

Vocal Tics:

Simple—throat clearing, coughing, sniffing, grunting, yelping, barking

Complex—saying words or phrases that do not make sense in a given situation, coprolalia (saying obscene or socially unacceptable words)

Many people with TS also have one or more of the following problems:

Diagnosis

The doctor will ask about your symptoms and medical history, and perform a physical exam. TS is diagnosed by observing the symptoms and reviewing when they began and how they progressed. There are no blood or neurological tests to diagnose TS. Some doctors may order an MRI scan, CT scan, EEG, or blood tests to rule out other disorders.

Treatment

Most people with TS do not need medical treatment. Two-thirds of people with TS either remit or have very mild symptoms, and 1/3 have significant symptoms. For people whose tics disrupt daily activities, there are treatments that can help manage symptoms. The most common is medication. However, the side effects may limit the effectiveness of medications. No single treatment is helpful for all people with TS, and treatment may not completely eliminate symptoms.

Medications

  • Several medications can help control tics, including clonidine (Catapres), pimozide (Orap), risperidone (Risperdal), and haloperidol (Haldol).
  • Obsessive-compulsive symptoms may be treated with Prozac, Anafranil, Zoloft, or other similar medications.
  • Symptoms of ADHD may be treated with stimulants such as Ritalin, Cylert or Dexedrine, or with tricyclic antidepressants.

Other Treatments

  • Behavior therapy can help people with TS learn to substitute their tics with other movements or sounds that are more acceptable.
  • Cognitive behavioral therapy can help reduce obsessive-compulsive symptoms.
  • Relaxation, biofeedback, and/or exercise can reduce the stress that often exacerbates symptoms.
  • Psychotherapy can help people with TS and their families cope with the disorder.

Prevention

There are currently no known ways of preventing TS.