One of the most common and least understood neurobiological disorders, Tourette syndrome is an inherited disorder that affects about 200,000 Americans. Males are affected three to four times as often as females.
Symptoms usually appear between the ages of four and eight, but in rare cases may emerge in the late teenage years. The symptoms include motor and vocal tics — repetitive, involuntary movements or utterances that are rapid, sudden and persist for more than one year. The types of tics may change frequently and increase or decrease in severity over time. In roughly one-half of individuals, this disorder lasts a lifetime, but the remaining patients may experience a remission or decrease in symptoms as they get older. A high percentage of people with Tourette syndrome also have associated conditions, such as problems with learning, difficulties with attention, obsessive thoughts and compulsive rituals. Often these manifestations are more troublesome to individuals than the tics themselves, so physicians must consider them when choosing a treatment regimen.
Tourette syndrome is inherited and seems to result from abnormal activity in a brain system called the basal ganglia. Research suggests that genes associated with Tourette’s, perhaps together with in utero or early environmental conditions, cause abnormalities in basal ganglia development or excesses in certain chemicals, including the neurotransmitter dopamine.
The majority of people with Tourette syndrome are not significantly disabled by symptoms, so they do not require medication. However, antipsychotics and SSRIs, as well as drugs to control tics, nausea, high blood pressure, seizures, or anxiety, are available to help control symptoms when they interfere with functioning. Stimulant medications such as methylphenidate and dextroamphetamine, which are prescribed for attention deficit hyperactivity disorder (ADHD), have been reported to improve attention and decrease tics in Tourette syndrome. For obsessive-compulsive symptoms that interfere significantly with daily functioning, SSRIs, antidepressants, and related medications may be prescribed.
Medication dosages that achieve maximum control of symptoms vary for each person and must be gauged carefully by a doctor. The medicine is administered in small doses, with gradual increases to the point where there is maximum alleviation of symptoms with minimal side effects. Some of the undesirable reactions to medications are weight gain, muscular rigidity, fatigue, motor restlessness, and social withdrawal. Most of these side effects can be reduced with specific medications. Other side effects, such as depression and cognitive impairment, can be alleviated with dosage reduction or a change of medication.
Other types of therapy also are helpful. Behavioral therapies, such as those used to treat similar disorders that emerge in childhood, have been receiving more attention. Aimed at training circuits to control the specific behavior related to the tic, these therapies have proven to be highly effective in reducing the severity of tics in some subtypes of Tourette syndrome. Psychotherapy and counseling can assist people with this disorder, as well as providing coping mechanisms for family members.