Tourette syndrome

Definition

Tourette (too-RET) syndrome is a neurological disorder in which you display unusual movements or make sounds over which you may have little or no control (tics). For instance, you may repeatedly blink your eyes, shrug your shoulders or jerk your head. In some cases, you might blurt obscenities.

Signs and symptoms of Tourette syndrome usually begin in childhood, typically showing up between ages 7 and 10. Males are about three to four times more likely than females to develop Tourette syndrome.

Although there's no cure, you can live a normal life span with Tourette syndrome, and many people with Tourette don't need treatment when symptoms aren't troublesome. Children often outgrow Tourette syndrome after adolescence.

Symptoms

Tics — sudden, brief, intermittent movements or sounds — are the hallmark sign of Tourette syndrome. Symptoms range from mild to severe and debilitating.

Tics are classified as either:

• Simple tics, which are sudden, brief and repetitive and involve a limited number of muscle groups
• Complex tics, which are distinct, coordinated patterns of movements involving several muscle groups

Tics involving movement (motor tics) — often facial tics, such as blinking — usually begin before vocal tics do. But the spectrum of tics that people experience is diverse, and there's no typical case.

Tics can vary in type, frequency and severity over time. They may worsen during periods of stress and anxiety, fatigue, illness, or excitement. They can occur during sleep. You'll likely experience an urge, called a premonitory urge, before the onset of motor or vocal tics. A premonitory urge is an uncomfortable bodily sensation, such as an itch, a tingle or tension. Expression of the tic brings relief.

Different tics may develop over time. Tourette symptoms are usually at their worst during the teenage years and sometimes improve during the transition to adulthood.

With great effort, some people with Tourette syndrome can sometimes temporarily stop a tic or hold back tics until they find a place where it's less disruptive to express them.

When to see a doctor
If you notice your child displaying unusual movements or behavior, schedule an appointment with your pediatrician. Not all tics indicate that someone has Tourette syndrome.

Many children develop tics that last a few weeks or months and then go away on their own. But whenever a child shows unusual behavior, it's important to have a medical evaluation to identify the cause and rule out serious health problems.

Causes

The exact cause of Tourette syndrome isn't known, and there's no known way to prevent it. Tourette is a complex syndrome, likely caused by a combination of genetic and environmental factors. Theories about the causes of Tourette include:

• Genetics. Tourette syndrome may be an inherited disorder. The specific genes involved in Tourette syndrome are still being defined, although one genetic mutation has been identified as a rare cause of Tourette syndrome.
• Brain abnormalities. Certain chemicals in the brain that transmit nerve impulses (neurotransmitters) may play a role, including dopamine and serotonin.

Risk factors

Having a family history of Tourette syndrome or other tic disorders may increase the risk of developing Tourette syndrome.

Complications

People with Tourette syndrome have a normal life span and often lead a healthy, active life. However, having Tourette syndrome may increase the risk of learning, behavioral and social challenges, which can mar self-image.

In addition, having Tourette syndrome means you're likely to have other related conditions, such as:

• Attention-deficit/hyperactivity disorder (ADHD)
• Obsessive-compulsive disorder
• Learning disabilities
• Sleep disorders
• Depression
• Anxiety disorders

Preparing for your appointment

You're likely to start by seeing your family doctor or pediatrician. However, in some cases when you call to set up an appointment, you may be referred immediately to a doctor who specializes in conditions of the nervous system (neurologist).

Here's some information to help you prepare for your appointment.

What you can do

• Write down any symptoms you or your child is experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
• Write down key personal information, including any major stresses or recent life changes.
• Make a list of all medications, vitamins or supplements you or your child is taking.
• Write down questions to ask your doctor.
• Make a video recording, if you have a video camera, of a typical tic to show the doctor.

Preparing a list of questions for your doctor will help you make the most of your time together. List your questions from most important to least important. For Tourette syndrome, some basic questions to ask your doctor include:

• Is this condition likely temporary or chronic?
• What is the best course of action?
• Are there any brochures or other printed material that I can take home with me? What Web sites do you recommend visiting?
• What family and patient support groups are there in my area for people with this diagnosis?

Don't hesitate to ask other questions during your appointment anytime you don't understand something or need more information.

What to expect from your doctor
Your doctor is likely to ask you a number of questions, including:

• When did the symptoms begin?
• Have the symptoms been continuous or occasional?
• How severe are the symptoms?
• What, if anything, seems to improve the symptoms?
• What, if anything, appears to worsen the symptoms?

Tests and diagnosis

There's no specific test that can diagnose Tourette syndrome. Instead, doctors must rely on the history of the person's symptoms to diagnose the disorder.

The Diagnostic and Statistical Manual of Mental Disorders (DSM) determines the criteria for a diagnosis of Tourette syndrome. Published by the American Psychiatric Association, the DSM is used by mental health professionals to diagnose certain conditions and by insurance companies to reimburse for treatment.

The criteria to diagnose Tourette syndrome include:

• Both motor tics and vocal tics must be present, although not necessarily at the same time.
• Tics occur several times a day, nearly every day or intermittently, for more than a year. There must not be a break in tics for more than a three-month period.
• The onset of tics occurs before age 18.
• Tics aren't caused by medications, other substances or another medical condition.

Diagnosis of Tourette syndrome may be delayed because families and even doctors are sometimes unfamiliar with the symptoms or the symptoms may mimic other problems. Eye blinking may be initially associated with vision problems, for instance, while sniffing may be attributed to allergies.

Because tics and movement problems can be the result of other serious health conditions, your doctor may suggest having tests to rule out other problems. These tests include blood tests or neuroimaging studies, such as magnetic resonance imaging (MRI).

Treatments and drugs

There's no cure for Tourette syndrome. Treatment is intended to help control tics that interfere with everyday activities and functioning. When tics aren't severe, treatment may be unnecessary.

Medications
No medication is helpful to everyone with Tourette syndrome, none completely eliminates symptoms, and they all have side effects to be weighed against the benefits. However, some medications can be used to help control or minimize tics or to control symptoms of related conditions, such as attention-deficit/hyperactivity disorder (ADHD) or obsessive-compulsive disorder (OCD). These may include:

• Drugs that block or deplete the neurotransmitter dopamine in the brain, such as fluphenazine or pimozide (Orap). Used to control tics, these medications may have side effects such as weight gain and a dulling of the mind.
• Botulinum Toxin Type A (Botox) injections. For simple or vocal tics, an injection into the affected muscle may help relieve the tic.
• Stimulant medications, such as methylphenidate (Concerta, Ritalin, others) and dextroamphetamine (Dexedrine, others). These are used to help increase attention and concentration for people with ADHD.
• Central adrenergic inhibitors, such as clonidine (Catapres) or guanfacine (Tenex). Typically prescribed for high blood pressure, these drugs may help control behavioral symptoms, such as impulse control problems and rage attacks. Side effects may include sleepiness.
• Antidepressants, such as fluoxetine (Prozac, Sarafem, others). These may help control the symptoms of OCD.

Therapies

• Psychotherapy. Psychotherapy can be helpful for two reasons. It can help with accompanying problems, such as ADHD, obsessions, depression and anxiety. Therapy can also help people cope with Tourette syndrome.
• Deep brain stimulation. For debilitating tics that don't respond to other treatment, deep brain stimulation (DBS) may help. DBS consists of implanting a battery-operated medical device (neurostimulator) in the brain to deliver electrical stimulation to targeted areas that control movement. Further research is needed to determine whether DBS is beneficial for people with Tourette syndrome.

Coping and support

Your self-esteem may suffer as a result of Tourette syndrome. You may be embarrassed about your tics. You may hesitate to engage in social activities, such as dating or going out in public. As a result, you're at increased risk of depression and substance abuse.

If you have Tourette syndrome, here are tips for coping with your disorder:

• Remember that tics usually get better as you get older. Tics usually reach their peak in the early teens to midteens.
• Reach out to others dealing with Tourette syndrome for support and information.

Children with Tourette syndrome
School may pose special challenges for children with Tourette syndrome.

To help your child:

• Get informed. Learn as much as you can about Tourette syndrome. Talk to your doctor about any questions you have.
• Be your child's advocate. Help educate teachers, school bus drivers and others with whom your child interacts regularly.
• Find a support group. To help you cope, ask your doctor for the names of local Tourette syndrome support groups. If there aren't any, consider starting one.