Tourette's Disorder

A neurological disorder that affects men four times as often as it does women, Tourette's disorder causes patients to have involuntary tics and movements. These can sometimes manifest as uncontrollable shouts or vocalizations.

Definition

Tourette's disorder, also referred to as Tourette's syndrome, is an inherited neurological disorder characterized by multiple tics that take the form of involuntary movements and uncontrollable vocalizations. A tic is a sudden, rapid, recurrent, nonrhythmic motor movement or vocalization. To be diagnosed with Tourette's disorder, a person must display multiple motor and one or more vocal tics, and these tics must have persisted for more than one year since their first onset.

The symptoms of Tourette's disorder generally appear before the individual is 18 years of age. The age of onset is typically between four and six years, and symptoms can reach their peak between the ages of 10 and 12. Tourette's disorder can affect people of all ethnic groups, but males are affected 3 to 4 times more often than females. It is estimated that three of every 1,000 people in the United States have Tourette's disorder.

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

Studies show that correct diagnosis of Tourette's is frequently delayed after the start of symptoms because many physicians may not be familiar with the disorder. The behavioral symptoms and tics are easily misinterpreted, often causing children with Tourette's disorder to be misunderstood at school, at home, and even in the doctor's office. Parents, relatives, and peers who are unfamiliar with the illness may incorrectly attribute the tics and other symptoms to psychological problems, thereby increasing the social isolation of those with the disorder. Because tics can wax and wane in severity and can also be suppressed in some cases, they are often absent during doctor visits, which further complicates making a diagnosis.

In many cases, parents, relatives, friends, and even the patients themselves become aware of the disorder based on information they have heard or read in the popular media.

Symptoms

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, current research points to abnormalities in certain brain regions (including the basal ganglia, frontal lobes, and cortex), the circuits that interconnect these regions, and the neurotransmitters (dopamine, serotonin, and norepinephrine) 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; 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 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 all associated with worse tic severity.

Treatments

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 persons 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. In addition, all medications have side effects.

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 very 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.

Recently scientists have discovered that long-term use of neuroleptic drugs may cause an involuntary movement disorder called tardive dyskinesia. However, this condition usually disappears when medication is discontinued. These side effects can be reduced by drugs commonly used to treat Parkinson's disease. Other side effects such as fatigue, depression, anxiety, weight gain, and difficulties in thinking clearly may be more troublesome.

Clonidine and guanfacine, both antihypertensive drugs, are also used in the treatment of 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 also 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.

Other types of 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. Larger and more definitive NIH-funded studies are underway.

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

What is the prognosis?

Although there is no cure for Tourette's disorder, the condition in many individuals improves as they mature. Individuals with Tourette's can expect to live a normal life span. Although the disorder is generally lifelong and chronic, it is not a degenerative condition. Tourette's does not impair intelligence. 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.

What is the best educational setting for children with Tourette's disorder?

Although students with Tourette's often function well in the classroom, it is thought that many may have some kind of 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, Fourth Edition
  • American Psychiatric Association, Diagnostic and Statistical Manual of Mental Disorders, Fifth 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 04/18/2017