What is Tourette syndrome?
Tourette syndrome (TS) is a neurological disorder that may cause sudden unwanted and uncontrolled rapid and repeated movements or vocal sounds called tics. TS is one of a group of disorders of the developing nervous system called tic disorders.
The motor (involving body movement) or vocal (involving sounds you make) tics of TS come and go over time, varying in type, frequency, location, and severity. If you have tics, you cannot stop your body from having them. The first symptoms usually occur between the ages of 5 and 10 years, generally in the head and neck area. These may progress to include the muscles in your torso, arms, and legs. Motor tics generally occur before the development of vocal tics.
Tourette syndrome affects more boys than girls. Most people with TS experience their worst tic symptoms in their early teens, but tics typically lessen and become controlled by the late teens to early 20s. For some people, TS can be a chronic condition with symptoms that last into adulthood. In some cases, tics may worsen in adulthood. TS is not a degenerative condition (one that continues to get worse) and individuals with TS have a normal life expectancy.
Classification of tics and symptoms
If you have TS, you may experience simple or complex motor tics. They may range from very mild to severe, although most cases are mild.
Simple tics are sudden, brief, repetitive movements that involve a few muscle groups. They are more common than complex tics. Simple tics often precede complex tics.
Simple motor tics include:
- Eye blinking and other eye movements
- Facial grimacing
- Shoulder shrugging
- Head or shoulder jerking
Simple vocal tics include:
- Repetitive throat clearing
Complex tics are distinct, coordinated patterns of movement involving several muscle groups in different parts of the body.
Complex motor tics might include facial grimacing combined with a head twist and a shoulder shrug. Other complex motor tics may appear purposeful, including:
- Sniffing or touching an object
Complex vocal tics may include:
- Repeating one's own words or phrases
- Repeating others' words or phrases (echolalia)
- Using vulgar, obscene, or swear words (coprolalia)
Some of the most dramatic and disabling tics may include motor movements that result in self-harm such as punching yourself in the face or vocal tics such as echolalia or swearing. Some tics are preceded by an urge or sensation in the affected muscle group (premonitory urge). You may feel like you have to complete a tic in a certain way or a certain number of times to relieve the urge or decrease the sensation.
Your tics may worsen with excitement or anxiety and get better during calm, focused activities. Certain physical experiences can trigger or worsen tics; for example, tight collars may trigger neck tics. Hearing another person sniff or clear the throat may trigger similar sounds. Tics do not go away during light sleep but are often significantly diminished; they go away completely in deep sleep.
Although the symptoms of TS are unwanted and unintentional (involuntary), some people can suppress or otherwise manage their tics to minimize their impact on functioning. However, people with TS often report a substantial buildup in tension when suppressing their tics to the point where they feel that the tic must be expressed (against their will). Tics in response to an environmental trigger can appear to be voluntary or purposeful but are not.
Disorders associated with TS
Like many other individuals with TS, you may experience additional co-occurring neurobehavioral problems (how the brain affects emotion, behavior, and learning) that often cause more impairment than the tics themselves. Although most people with TS experience a significant decline in motor and vocal tics in late adolescence and early adulthood, the associated neurobehavioral conditions may continue into adulthood.
The most common co-occurring conditions include:
- Attention Deficit Hyperactivity Disorder (ADHD)—People with TS may have problems with concentration, hyperactivity, and impulsivity.
- Obsessive Compulsive Disorder or Behaviors (OCD/OCB)—Unwanted thoughts, ideas, or sensations (obsessions) may make someone with TS feel the need to perform behaviors repeatedly or in a certain way (compulsions). Repetitive behaviors can include handwashing, checking things, and cleaning, and can significantly interfere with daily life.
- Anxiety—People with TS, along with others, may have a fear, uneasiness, or apprehension about a situation or event that may have an uncertain ending.
- Learning disabilities—Problems with reading, writing, and arithmetic that are not related to intelligence are seen in some individuals with TS.
- Behavioral or conduct issues—Common issues include aggression, anger management problems, and problems adjusting emotionally and socially.
- Problems falling or staying asleep—Sleep disturbances may include difficulty falling and staying asleep and excessive daytime sleepiness.
- Social skills deficits and social functioning difficulties—People may have trouble with social skills and with maintaining social relationships.
- Sensory processing issues—Some people with TS have difficulty organizing and responding to sensory information related to touch, taste, smells, sounds, or movement.
Although students with TS often function well in the regular classroom, ADHD, learning disabilities, obsessive-compulsive symptoms, and frequent tics can greatly interfere with academic performance or social adjustment. After a comprehensive assessment, students should have access to an educational setting that meets their individual needs. Students may require tutoring, smaller or special classes, private study areas, exams outside the regular classroom, other individual performance accommodations, and in some cases special schools.
Who is more likely to get Tourette syndrome?
Most cases of TS involve the interaction of multiple gene variations and environmental factors.
Although the cause of TS is unknown, current research points to abnormalities in:
- Certain brain regions, including the basal ganglia, frontal lobes, and cortex
- Circuits that connect these regions
- Neurotransmitters (dopamine, serotonin, and norepinephrine) responsible for communication between nerve cells (neurons)
Changes (mutations) in one or more genes have been shown to be involved with TS. A small number of people with TS have mutations involving the SLITRK1 gene, which affects how neurons grow and connect with one another. Abnormalities in the NRXN1 and CNTN6 genes, which also regulate the normal formation of these nerve connections, also may play a role in TS. Scientists continue to look for other genes involved with TS. Although there may be a few genes with substantial effects, it is also possible that many genes with smaller effects and environmental factors may play a role in the development of TS.
It is important to understand that genetic tendency may not necessarily result in TS; instead, it may express itself as a milder tic disorder or as obsessive-compulsive behaviors. It is also possible that children who inherit the gene abnormality will not develop any TS symptoms.
Genetic studies also suggest that some forms of ADHD and OCD are genetically related to TS, but there is less evidence for a genetic relationship between TS and other neurobehavioral problems that commonly co-occur with TS.
Gender also plays an important role in TS gene expression. At-risk males are more likely to have tics and at-risk females are more likely to have obsessive-compulsive symptoms.
Genetic counseling of people with TS should include a full review of all potentially hereditary conditions in the family.
How is Tourette syndrome diagnosed and treated?
To diagnose TS, a doctor will ask if you have:
- Presence of both motor and vocal tics that occur several times a day, every day or intermittently for at least 1 year
- Onset of tics before age 18
- Tics not caused by medications, other substances, or medical conditions
Common tics are often diagnosed by a primary care provider, pediatrician, or mental healthcare specialist. Atypical symptoms (different from classical symptoms) or atypical presentations (for example, symptoms that begin in adulthood) may require additional expertise for diagnosis.
There are no blood, laboratory, or imaging tests needed for diagnosis. In rare cases, neuroimaging studies, such as magnetic resonance imaging (MRI) or computerized tomography (CT), electroencephalogram (EEG) studies, or certain blood tests may be used to rule out other conditions that might be confused with TS.
It may take some time to receive a formal diagnosis of TS. Families and physicians unfamiliar with the disorder might think mild and even moderate tic symptoms may be insignificant, a part of a developmental phase, or the result of another condition. For example, some parents may think that eye blinking is related to vision problems or that sniffing is related to seasonal allergies.
Currently, there is no cure for TS but treatments are available to help manage some symptoms.
If tic symptoms are mild and do not cause impairment, you may not need treatment. If symptoms interfere with daily functioning, there are effective medications and other treatments. Some medications may have or cause side effects and should be carefully managed by your physician or healthcare provider.
- Medications that block dopamine (drugs that may be used to treat psychotic and non-psychotic disorders) are the most consistently useful medications to suppress tics (for example, haloperidol and pimozide).
- Alpha-adrenergic agonists such as clonidine and guanfacine are used primarily for hypertension (high blood pressure) but are also used to treat tics.
- Stimulant medications such as methylphenidate and dextroamphetamine can lessen ADHD symptoms in people with TS without causing tics to become more severe. Some studies show the short-term use of these drugs can help children with TS who also have ADHD.
- Antidepressants, specifically, serotonin reuptake inhibitors (clomipramine, fluoxetine, fluvoxamine, paroxetine, and sertraline) can help some people control symptoms of depression, OCD, and anxiety.
Unfortunately, there is no one medication that is helpful to all people with TS, nor does any medication completely eliminate symptoms.
Other therapies and treatments can include:
- Behavioral treatments such as awareness training and competing response training can be used to reduce tics. A recent National Institutes of Health-funded, multi-center randomized control trial called Cognitive Behavioral Intervention for Tics (CBIT) showed that training to voluntarily move in response to a premonitory urge can reduce tic symptoms. Other behavioral therapies, such as biofeedback or supportive therapy, have not been shown to reduce tic symptoms. However, supportive therapy can help a person with TS better cope with the disorder and deal with the secondary social and emotional problems that can occur.
- Psychotherapy can help individuals cope with the disorder and deal with accompanying problems or conditions, including ADHD, depression, anxiety, and OCD.
What are the latest updates on Tourette syndrome?
The National Institute of Neurological Disorders and Stroke (NINDS) and other components of the National Institutes of Health (NIH)—such as the National Institute of Mental Health, the Eunice Kennedy Shriver National Institute of Child Health and Human Development, the National Institute on Drug Abuse, and the National Institute on Deafness and Other Communication Disorders—support research relevant to TS, either at NIH laboratories or through grants to major research institutions across the country.
Another component of the Department of Health and Human Services, the Centers for Disease Control and Prevention (CDC), funds professional education programs as well as TS research.
Knowledge about TS comes from studies across numerous medical and scientific disciplines, including genetics, neuroimaging, neuropathology, clinical trials (medication and non-medication), epidemiology, neurophysiology, neuroimmunology, and descriptive/diagnostic clinical science.
- Genetic studies—Currently, NIH-funded scientists are conducting a variety of large-scale genetic studies involving TS. Understanding the genetics of TS genes may strengthen clinical diagnosis, improve genetic counseling, lead toward a better understanding of its causes, and provide clues for more effective therapies.
- Neurostimulation—NINDS-funded research is testing the effectiveness and safety of deep brain stimulation (DBS) for treating tics and co-occurring conditions, such as obsessive-compulsive behaviors, in individuals with TS who do not respond well to medications and behavioral therapy. DBS uses a surgically implanted, battery-operated medical device to deliver electrical stimulation to specific areas in the brain that control movement, which blocks the abnormal nerve signals that cause symptoms.
- Neuroimaging studies—Advances in imaging technology and an increase in trained scientists have led to an increasing use of novel and powerful techniques to identify brain regions, circuitry, and neurochemical factors important in TS and related conditions, such as ADHD and OCD.
- Neuropathology (the study of nervous system diseases)—There has been an increase in the number and quality of donated brains from people with TS available for research. This increase, coupled with advances in neuropathological techniques, has led to initial findings with implications for neuroimaging studies and animal models of TS.
- Clinical trials—Several clinical trials in TS recently have been completed or currently are underway. These include studies of stimulant treatment of ADHD in TS and behavioral treatments for reducing tic severity in children and adults. Smaller trials of novel approaches to treatment such as dopamine agonists and glutamatergic medications also show promise. Neurostimulation treatments mentioned above such as DBS and noninvasive transcranial magnetic stimulation in children and adults are also ongoing.
- Epidemiology and clinical science—Careful epidemiological studies (those that track the pattern or incidence of a disease) now estimate the prevalence of TS to be substantially higher than previously thought, with a wider range of severity. Also, clinical studies are providing new findings regarding TS and co-existing conditions. These include subtyping studies of TS and OCD, an examination of the link between ADHD and learning problems in children with TS, and a new appreciation of sensory tics. One of the most important and controversial areas of TS science involves the relationship between TS and autoimmune brain injury associated with group A beta-hemolytic streptococcal infections or other infectious processes. There are many epidemiological and clinical investigations currently underway in this area.
You may find more information about research on TS by using NIH RePORTER, a searchable database of current and past research projects supported by NIH and other federal agencies. RePORTER also includes links to publications and resources from these projects.
How can I or my loved one help improve care for people with Tourette syndrome?
Consider participating in a clinical trial so clinicians and scientists can learn more about TS. Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease.
All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.
For information about participating in clinical research visit NIH Clinical Research Trials and You. Learn about clinical trials currently looking for people with TS at Clinicaltrials.gov, a searchable database of current and past federal and private clinical trials.
Where can I find more information about Tourette syndrome?
Information may be available from the following resources:
Child Neurology Foundation
Tourette Association of America