Gilles de la Tourette syndrome

Answer 1

Definition

Gilles de la Tourette syndrome is a disorder of the nervous system that causes a person to make repeated and uncontrolled (involuntary) rapid movements and sounds (vocalizations) called tics. The disorder is commonly called Tourette syndrome.

See also:

• Chronic motor tic disorder
• Facial tics
• Transient tic disorder

Alternative Names

Tourette syndrome

Causes, incidence, and risk factors

Tourette syndrome is named for Georges Gilles de la Tourette, who first described this disorder in 1885. There is strong evidence that Tourette syndrome is passed down through families, although the gene has not yet been found.

The syndrome may be linked to problems in certain areas of the brain, and the chemical substances (dopamine, serotonin, and norepinephrine) that help nerve cells talk to one another.

Tourette syndrome can be either severe or mild. About 10% of Americans have a mild tic disorder, but far fewer have more severe forms of Tourette syndrome. Many people with very mild tics may not be aware of them and never seek medical help.

Tourette syndrome is four times as likely to occur in boys as in girls.

Symptoms

Most affected people first notice symptoms of Tourette syndrome during childhood, between ages 7 and 10.

Symptoms of Tourette syndrome can range from almost unnoticeable minor movements (such as grunts, sniffling, or coughing) to continuing, uncontrollable movements and sounds (vocalizations).

The symptoms tend to get better and worse. Symptoms usually get worse before the mid-teen years.

The most common first symptom is a facial tic. Other tics may follow. A tic is a sudden, rapid, repeated movement or voice sound (vocalization). Tics can include:

• Arm thrusting
• Eye blinking
• Jumping
• Kicking
• Repeated throat clearing or sniffing
• Shoulder shrugging

Tics may occur many times a day, but they tend to improve or get worse at different times. The tics may change with time.

Contrary to popular belief, use of curse words or other inappropriate words or phrases (coprolalia) occurs in only a small number of patients.

Many patients say that the tics are not totally out of their control (involuntary), but that "things just would not feel right" if they did not do them. This is what makes Tourette syndrome different from obsessive-compulsive disorder (OCD) -- people with OCD feel as though they have to do the behaviors.

Many people with the disorder can suppress the tic for periods of time, but find that when it is allowed to occur after that time, it is more dramatic for a few minutes.

Signs and tests

There are no lab tests to diagnose Tourette syndrome. However, a health care provider should do an examination to rule out other causes of these symptoms.

To be diagnosed with Tourette syndrome, a person must:

• Have had many motor tics and one or more vocal tics at some time, although not necessarily at the same time
• Have tics that occur many times a day, nearly every day or on and off, for a period of more than 1 year; during this period, there must not be a tic-free period of more than 3 months in a row
• Have started the tics before age 18
• Have no other brain problem that could be a likely cause of the symptoms

Treatment

Many patients with Tourette syndrome have very minor symptoms. In this case, they are usually not treated, because the side effects of the medications may be worse than the symptoms of the condition.

Drugs used to treat tics include dopamine blockers, such as fluphenazine, haloperidol, pimozide and risperidone. These medicines can help control or reduce tics, but they have side effects such as movement disorders and cognitive dulling. Antiseizure medications are also used sometimes.

A blood pressure medicine called clonidine has been shown to help control tics. Another drug commonly used is tetrabenazine, but this drug is also linked to movement disorders as well as depression. Many other treatments have been tried with little or no improvement.

Deep brain stimulation (DBS) has given encouraging results both for the main symptoms of Tourette syndrome and for the associated obsessive-compulsive mannerisms.

Support Groups

Tourette Syndrome Association - www.tsa-usa.org

Expectations (prognosis)

Most patients improve in early adulthood. Although 1 in 4 patients may be symptom-free for a few years, only 8% of patients have symptoms completely go away without returning.

People with Tourette syndrome have a normal life expectancy.

Complications

Conditions that may occur in people who have Tourette syndrome include:

• Anger control issues
• Attention deficit hyperactivity disorder (ADHD)
• Impulsive behavior
• Obsessive-compulsive disorder
• Poor social skills

These conditions need to be diagnosed and treated.

Calling your health care provider

Make an appointment with your health care provider if you have tics that are severe or persistent, or if they interfere with your daily life.

Prevention

There is no known prevention.

References

Jankovic J. Movement disorders. In: Goetz, CG, ed. Textbook of Clinical Neurology. 3rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 34.

Lang A. Other movement disorders. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 434.

Kuhn JTO. Deep brain stimulation for psychiatric disorders. Dtsch Arztebl Int. 2010. 107(7): 105-113.

Answer 2

Definition

Gilles de la Tourette syndrome is a condition that causes people to make repeated, quick movements or sounds that they cannot control. These movements or sounds are called tics.

The condition is commonly called Tourette syndrome.

See also:

• Chronic motor tic disorder
• Facial tics
• Transient tic disorder

Alternative Names

Tourette syndrome

Causes, incidence, and risk factors

Tourette syndrome is named for Georges Gilles de la Tourette, who first described this disorder in 1885. There is strong evidence that Tourette syndrome is passed down through families, although the gene has not yet been found.

The syndrome may be linked to problems in certain areas of the brain. It may have to do with chemical substances (dopamine, serotonin, and norepinephrine) that help nerve cells talk to one another.

Tourette syndrome can be either severe or mild. About 10% of Americans have a mild tic disorder. Far fewer people have more severe forms of Tourette syndrome. Many people with very mild tics may not be aware of them and never seek medical help.

Tourette syndrome is four times as likely to occur in boys as in girls.

Symptoms

Most people with Tourette syndrome first notice symptoms during childhood, between ages 7 and 10.

The most common first symptom is a tic of the face, but other tics may follow. A tic is a sudden, fast, repeated movement or sound.

Symptoms of Tourette syndrome can range from tiny, minor movements (such as grunts, sniffling, or coughing) to constant movements and sounds that can't be controlled.

Tics can include:

• Arm thrusting
• Eye blinking
• Jumping
• Kicking
• Repeated throat clearing or sniffing
• Shoulder shrugging

Tics may occur many times a day, but they tend to improve or get worse at different times. The tics may change with time. Symptoms usually get worse before the mid-teen years.

Contrary to popular belief, only a small number of patients use curse words or other inappropriate words or phrases (coprolalia).

Tourette syndrome is different from obsessive-compulsive disorder (OCD). People with OCD feel as though they have to do the behaviors.

Many people with Tourette syndrome can stop doing the tic for periods of time. However, they find that the tic is stronger for a few minutes after they allow it to start again. Often the tic slows or stops during sleep.

Signs and tests

There are no lab tests to diagnose Tourette syndrome. However, a health care provider should do an examination to rule out other causes of the symptoms.

To be diagnosed with Tourette syndrome, a person must:

• Have had many motor tics and one or more vocal tics, although these tics may not have occurred at the same time
• Have tics that occur many times a day, nearly every day or on and off, for a period of more than 1 year. During this period, there must not have been a tic-free period of more than 3 months in a row.
• Have started the tics before age 18
• Have no other brain problem that could be a likely cause of the symptoms

Treatment

Many patients with Tourette syndrome who have very minor symptoms are not treated, because the side effects of the medications may be worse than the symptoms of Tourette syndrome.

Drugs used to treat tics include:

• Antiseizure medications
• Blood pressure medicine called clonidine
• Botulinum toxin injections (can control some motor tics)
• Dopamine blockers, such as fluphenazine, haloperidol, pimozide and risperidone (can help control or reduce tics, but they have side effects)

Deep brain stimulation has shown promise for treating both the main symptoms of Tourette syndrome and the obsessive-compulsive behaviors.

Support Groups

Tourette Syndrome Association - www.tsa-usa.org

Expectations (prognosis)

Symptoms usually peak during the teenage years and then improve in early adulthood. Although 1 in 4 patients may be symptom-free for a few years, only 8% of symptoms go away without returning.

Patients usually have normal intelligence and live a normal-length life.

Complications

Conditions that may occur in people who have Tourette syndrome include:

• Anger control issues
• Attention deficit hyperactivity disorder (ADHD)
• Impulsive behavior
• Obsessive-compulsive disorder
• Poor social skills

These conditions need to be diagnosed and treated.

Calling your health care provider

Make an appointment with your health care provider if you have tics that are severe or persistent, or if they interfere with your daily life.

Prevention

There is no known prevention.

References

Gleason MM, Boris NW, Dalton R. Habit and tic disorders. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 23.

Jankovic J, Lang AE. Movement disorders: diagnosis and assessment. In: Bradley WG, Daroff RB, Fenichel GM, Jankovic J, eds. Neurology in Clinical Practice. 5th ed. Philadelphia, Pa: Butterworth-Heinemann Elsevier; 2008:chap 23.

Lang A. Other movement disorders. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 434.

Kurlan R. Clinical practice Tourette's Syndrome. N Engl J Med. 2010;363(24):2332-2338.

Reviewed By

Review Date: 02/05/2011

David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Joseph V. Campellone, MD, Division of Neurology, Cooper University Hospital, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.