Tourette's Syndrome

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What is Tourette's Syndrome (TS)?

Tourette's syndrome is a rare complex neuropsychiatric condition characterized by the presence of tics, which are usually associated with other disorders such as:

This tic disorder was first described by Georges Gilles de la Tourette in 1885.

The first signs and symptoms are seen most often around the ages of 6 to 8; however, in some cases the first signs are seen at an earlier age, and in other cases they start in adolescence.

What Causes Tourette's Syndrome?

Our present understanding is that Tourette's syndrome is a biological disorder of the brain, but the exact reasons for of the tics and the associated disorders that are often seen in persons with Tourette's syndrome are not clear.

Fortunately, Tourette's syndrome is not a fatal condition; hence, there are very few possibilities of performing autopsies on individuals with Tourette's syndrome. In the few autopsies reported most of the abnormalities were seen in an area deep in the brain, the basal ganglia, which is known to be strongly associated with the control of movement. This is an expected finding since this area of the brain is known to be abnormal in other conditions that are also associated with movement disorders not related to Tourette's syndrome. Recently, MRI studies of the brain in persons with Tourette's syndrome also have shown some abnormalities in this area of the brain.

Heredity

There is a familial incidence of Tourette's syndrome. First-degree relatives of persons with Tourette's syndrome more frequently have tics and obsessive compulsive disorders or attention deficit hyperactivity disorder (ADHD) than the general population. Also, twin studies have shown that identical twins (monozygotic twins) are five times more likely to both have Tourette's syndrome than in twins that are not identical (dizygotic twins). These observations suggest an autosomal dominant inheritance of the condition with variable penetrance.

However, in spite of this strong evidence of genetic involvement, at the present time, no gene has been identified as related to Tourette's syndrome. Moreover, other factors are certainly also responsible for the symptoms. For example, the severity of the syndrome in affected identical twins is not necessarily the same. For example, Tourette's syndrome is more severe in the twin who experienced greater perinatal complications.

Immune Disorders

The observation of tics developing after streptococcal infections motivated clinical trials to look at the role of autoimmune disorders as a cause of Tourette's syndrome. It is known that streptococcal infections can trigger, in certain individuals, autoimmune disorders that can attack and damage the basal ganglia, resulting in Sydenham chorea. This is a movement disorder characterized by multiple abnormal movements, including tics, as well as other behavioral issues such as obsessive compulsive disorders, which are also seen in people with Tourette's syndrome. Also clinical trials have looked at the role that pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) may play in the development and prognosis of Tourette's syndrome, but, presently, this is only a hypothesis and has not been proven.

Medications

Finally studies are inconclusive about the possible association between exposure to stimulants methylphenidate (Ritalin, Ritalin SR, Ritalin LA), amphetamines (Adderall) and certain other medications lamotrigine (Lamictal) and the precipitation of Tourette's syndrome.

Gender

It has also been found that Tourette's syndrome is more common in boys than in girls by a ratio of five to one.

Race/Ethnic Background

Tourette's syndrome has been described in people of many ethnic backgrounds. At the present time there is no indication that Tourette's syndrome is more frequent in any particular ethnic group.

What Are the Signs and Symptoms of Tourette's Syndrome?

Tics

The leading sign of Tourette's syndrome, and the most common reason for referral for consultation, is the presence of tics. However, it is worthwhile to mention that even though tics might be disabling, they are not necessarily, as will be discussed later on, the most disabling problem in persons with Tourette's syndrome.

Tics are repetitive, involuntary or semi-voluntary, short lasting, stereotyped movements (motor tics) or vocalizations (phonic tics), of sudden presentation, usually in clusters. There are many clinical varieties of tics that can affect any part of the body, but they are more common in the face, trunk, and shoulders.

Traditionally, tics have been divided in two main groups:

  1. motor tics, and
  2. vocal tics.

The motor tics are described as simple motor tics when they involve a single muscle, or complex motor tics when they consists of a more coordinated movement resembling a normal function.

Similarly, the vocal tics can be simple vocal tics when they consist of simple sounds or complex when the they consist in the production of words or sentences (complex phonic tics).

The following are examples of tics commonly seen in persons with Tourette's:

  • Simple motor tics include:
    • eye blinking,
    • shoulder rotation or elevation,
    • head jerking,
    • lip contractions,
    • closing of the eyes,
    • eyes rolling in the orbits,
    • torticollis (turning the neck to one side),
    • opening and closing of the mouth,
    • abdominal contractions, and/or
    • stretching of arms and legs.
  • Complex motor tics include:
    • jumping,
    • kicking,
    • touching objects,
    • retching,
    • trunk bending or rotation,
    • burping,
    • socially inappropriate movements,
    • obscene gestures, or
    • imitation of other peoples' gestures.
  • Simple phonic tics include:
    • grunting,
    • clearing throat,
    • coughing,
    • meaningless sounds or utterances.
  • Complex phonic tics include:
    • complex and loud sounds,
    • phrases out of context,
    • phrases with obscenities,
    • cursing,
    • repetition of other person's phrases.

Tics might be transiently suppressed by the individual. Additionally, tics may also be suppressed with tasks that require concentration or by distraction. For example, when a child with tics is watching TV or playing video games, the tics maybe suppressed to a minimum. This characteristic could lead a non-informed observer to believe that the movements are fully under the child's control. However, this is not the case. Even though the patient has some control, prolonged suppression of tics is usually associated with an unpleasant sensation that is relieved only by the tic. Voluntary suppression of tics is a very taxing task for the person with Tourette's syndrome.

Most individuals with Tourette's syndrome perceive some inner body sensation before the tic occurs. For example he/she may feel a burning or an itching of the eyes that is suppressed by moving the eyes, or a tickling in the throat that is relieved only by "clearing the throat". After this subjective feeling, the patient may need to repeat the tic several times until the unpleasant sensation is gone. In some individuals a non-well defined urge precedes the tic.

Stress, anxiety, fatigue and boredom can worsen the symptoms of a person with Tourette's syndrome.

Moreover, tics have a wax and wane quality. Tics have a tendency to cluster during certain hours and under certain circumstances rather than being present evenly throughout the day. Also, tics might not be seen for hours after a severe cluster.

Additionally, the quality, frequency and type of tics change during the evolution of the disease. Tics that were once frequently seen are suppressed and exchanged for other tics.

Usually, the first signs of disease start in childhood. The tics may increase in frequency and severity in adolescence and, even though the condition is chronic, there is a tendency to improve in adulthood. By age 18, 50 % of patients with Tourette's syndrome might be symptom-free; however, some individuals might see a recurrence of the symptoms later in life. Usually, simple motor tics are seen at an early age and precede verbal tics. Also, complex tics are first seen later in life.

Other Associated Conditions

Associated conditions have been reported in almost half of the children with Tourette's syndrome. The most common are attention deficit hyperactivity disorder (ADHD) and obsessive compulsive disorder (OCD). Both of these disorders can be observed before school age. It is not clear why these conditions are so frequently present. It is probable that they share a common pathological mechanism in the brain.

In addition, individuals with Tourette's syndrome may also have depression, anxiety, and other behavioral problems. In some cases, this may be attributed to being perceived as different or being rejected by peers.

Developmental disability is not a feature of Tourette's syndrome, however the presence of ADHD may disrupt learning, resulting in poor grades.

When to Seek Medical Care for Tourette's

The development of tics in an otherwise healthy child is an indication for consultation with a pediatrician.

If the tics do not improve or if there is any doubt about the diagnosis, the pediatrician probably will send the child to a specialist. In this case a professional with special knowledge in Tourette's syndrome would be the best choice. In some large academic centers it is possible to find specialized clinics, but since Tourette's syndrome is not common, this most likely is not a choice for most individuals.

Pediatric neurologists often specialize in Tourette's syndrome, and this would probably be the best choice for diagnostic opinions and treatment.

Depending upon the severity of the associated conditions the patient might need psychiatric consultation to help with appropriate treatment options for severe co-morbid conditions (ADHD, OCD, LD, mood disorder, severe aggression, sleep difficulties).

Depending upon the severity of the psychological problems, a consultation with a psychologist might be needed. Also, depending upon the severity of any associated learning disorders, a special individualized education plan (IEP) might be necessary.

Questions to Ask the Doctor about Tourette's

The first question should be the confirmation of the diagnosis. Since this is a chronic condition, and the doctor will be involved with the patient for a long period of time, it is important to know if the doctor has experience in dealing with Tourette's syndrome.

Next, it is important to know if the patient can benefit from the use of medications, and if so, how long the medication(s) should be used. It is also essential to ask about any possible side effects of the medications, for example if the medications are safe to take in pregnancy or if it is safe to drive while taking the medication(s).

Exams and Tests for Tourette's

The diagnosis of Tourette's syndrome is based on clinical information and a physical examination.

At the present time there is no test that will confirm the diagnosis. However, the physician might recommend some tests in certain cases just to rule out other possible diseases.

The Diagnostic and Statistical Manual for Mental Disorders, 4th edition (DSM-IV), a common source of reference for diagnostic purposes, established as a criteria for the diagnosis of Tourette's syndrome:

  • the presence of both multiple motor tics and one or more phonic tics that might be present at some time, although not necessarily concurrently.
  • The tics must occur many times a day (usually in bouts) nearly every day or intermittently over more than a year, during which time there must not have been a tic-free period of more that three consecutive months. The onset occurs before the age of 18 years. Also there may not be other explanation for the tics.

When the patient meets these criteria it is usually not necessary to perform other tests.

There are some scales, such as the Yale Global Tic Severity Scale (YGTSS), that may be helpful to determine the level of impairment and to evaluate treatment options.

Neuropsychological testing may be indicated only for children with school problems, otherwise this is not useful.

What Is the Treatment for Tourette's Syndrome?

The treatments that are available are all symptomatic, meaning that they are directed at improving the symptoms rather than eliminating the cause of the disease. No curative or preventative treatment is available.

The goal of the treatment should be to help the patient to live a normal life, with the understanding that, at the present time, the treatments available do not suppress all of the symptoms. Since associated conditions may be more disabling that the tics, the treatment should be tailored to the needs of the particular individual and directed to the most troublesome symptoms.

It should be noted that as Tourette's syndrome is a chronic condition and the symptoms naturally wax and wane, any apparent success of a treatment might be an expression of the natural evolution of the disease more so than the effect of the treatment.

In most instances treatment with medication is not necessary. However, if the severity of the symptoms affects the patient's social integration or the tics are very painful or result in self-injurious behavior, then a trial with medication might be indicated.

Generally, medications should be combined with behavioral approaches to decreasing stress and anxiety.

Several treatment options, including pharmacological and non-pharmacological therapies, are presented below.

What Are the Medications for Tourette's?

Treatment of Tics

The most effective medication for the suppression of tics is haloperidol (Haldol), a dopamine blocker medication originally approved for the treatment of psychiatric disorders. Unfortunately, this medication may result in a serious complication, tardive dyskinesia, which might be more disabling that the tics. Even though this complication has not been described in persons with Tourette's syndrome, the use of haloperidol is limited to the most serious cases.

Other medications in this group such as olanzapine (Zyprexa), risperidone (Risperdal), ziprasidone (Geodon), or aripiprazole (Abilify) might have less side effects than haloperidol (Haldol), but there is not enough clinical experience with these drugs in Tourette's syndrome, so their use is very limited.

Clonidine (Catapres) and guanfacine (Tenex), first introduced as cardiovascular medications, are effective in the treatment of tics and also in decreasing anxiety. These medications may be an acceptable first option in some patients.

Clonazepam (Klonopin) belongs to a group of medications (the benzodiazepines) that were first used because of their sedative and relaxing effect. From this group clonazepam can be effective in decreasing some tics and also in helping with anxiety disorders. Side effects such as sedation, weakness, and tiredness might be a limiting factor.

Botulinum toxin injections might be useful for certain disabling localized tics. The effect may only last for a few months, and repeated treatments might result in tolerance, rendering the drug ineffective after several applications.

Treatment of Attention Deficit Hyperactive Disorder (ADHD)

ADHD is not uncommon in children with Tourette's syndrome. Treating the deficit in attention as well as the hyperactivity with medications such as methylphenidate (Ritalin) or amphetamines (Adderall) might be very effective when accommodations in the school setting fail. There are some concerns with the use of these medications because, allegedly, they can produce or exacerbate existing tics. However, several studies have shown that their effects on tics is temporary even with continuous use. So if these medications are indicated the presence of tics is not an absolute contraindication to their use.

Treatment of Obsessive Compulsive Disorder (OCD)

As with tics and ADHD, the treatment of OCD depends on the severity of the clinical symptoms. If medications are needed to treat OCD the guidelines are the same as in persons without Tourette's syndrome.

When agents such as clomipramine (Anafranil), fluoxetine (Prozac), or risperidone (Risperdal) are used for the treatment of OCD the tics might also improve.

Non-Pharmacological Therapies for Tourette's

  • Habit Reversal Therapy, a form of behavior therapy for tics, has proven to decrease the frequency of tics.
  • Supportive therapy (guided imagery, role playing, deep breathing, yoga or tai chi for deep relaxation) including techniques for decreasing anxiety and stress can be extremely helpful in decreasing the severity and frequency of symptoms.
  • Additional counseling can help the patient to understand his/her condition, as well as to improve self-esteem and social adaptation.
  • There is no evidence that diets may improve the symptoms of Tourette's syndrome. People with Tourette's syndrome should be aware that some herbal products for weight loss may contain ingredients that could exacerbate the tics.
  • Furthermore, there is no evidence that dietary supplements can decrease the intensity of the symptoms.
  • Treatment with antibiotics, even in patients who have indications of past infection, is not indicated.
  • Repetitive transcranial magnetic stimulation has not been effective in relieving the symptoms associated with Tourette's syndrome.

Surgery for Tourette's Syndrome

Stereotactic neurosurgery is very rarely indicated for the treatment of tics or the symptoms of obsession and/or compulsions.

What Is the Follow-Up for Tourette's?

In those patients that are on mediation, follow-up care should include monitoring of the medication's side-effects and periodic taper/discontinuation under medical supervision to determine if the medications are still needed and if the dosage is effective.

Can You Prevent Tourette's?

There is no known prevention of this Tourette's syndrome. However, some of the psychological complications can be secondary to the social limitations imposed by the disease. Close monitoring of the patient for the early detection of emergent emotional disorders is very important. Also, education of the persons that relate to him/her (family members, teachers, classmates, friends) may also help to create a better environment for the child and prevent emotional issues.

What Is the Prognosis for Tourette's Syndrome?

Prognosis is good, as some individuals have improvement in symptoms either spontaneously or due to appropriate pharmacologic and behavioral treatment, and especially with successful management of situations that are likely to exacerbate tics (anxiety, stress).

The mortality rate is the same as in the general population.

Support Groups and Counseling for Tourette's

Participation in support groups can be extremely helpful for families and individuals with Tourette's syndrome. When associated symptoms such as attention deficit hyperactivity disorder, depression, or aggression occur, it is important to receive appropriate support services which include counseling to reduce stress and anxiety, as well as mental health services.

The national Tourette's Syndrome Association has chapters in most of the 50 states and also links internationally.

For individuals with associated Attention Deficit Hyperactivity Disorder the web site for CHADD.

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Reviewed on 5/15/2018
Sources: References

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