Tourette syndrome (TS) is a neurological disorder characterized by repetitive, stereotyped, involuntary movements and vocalizations called tics. The disorder is named for Dr. Georges Gilles de la Tourette, the pioneering French neurologist who in 1885 first described the condition in an 86-year-old French noblewoman.
Although the cause of TS is unknown, current research points to abnormalities in certain brain regions:
- basal ganglia
- frontal lobes
-the circuits that interconnect these regions
are responsible for communication among nerve cells.
Tics are classified as either simple or complex. Simple motor tics are sudden, brief, repetitive movements that involve a limited number of muscle groups. Some of the more common simple tics include:
- eye blinking
- other eye movements
- facial grimacing
- shoulder shrugging
- head or shoulder jerking Simple vocalizations might include repetitive throat-clearing, sniffing, or grunting sounds. Complex tics are distinct, coordinated patterns of movements involving several muscle groups. Complex motor tics might include facial grimacing combined with a head twist and a shoulder shrug. Other complex motor tics may actually appear purposeful, including sniffing or touching objects, hopping, jumping, bending, or twisting. Simple vocal tics may include throat-clearing, sniffing/snorting, grunting, or barking. More complex vocal tics include words or phrases. Perhaps the most dramatic and disabling tics include motor movements that result in self-harm such as punching oneself in the face or vocal tics including coprolalia (uttering socially inappropriate words such as swearing) or echolalia (repeating the words or phrases of others). However, coprolalia is only present in a small number (10 to 15 percent) of individuals with TS. Some tics are preceded by an urge or sensation in the affected muscle group, commonly called a premonitory urge. Some with TS will describe a need to complete a tic in a certain way or a certain number of times in order to relieve the urge or decrease the sensation.
TS is a diagnosis that doctors make after verifying that the patient has had both motor and vocal tics for at least 1 year. The existence of other neurological or psychiatric conditions can also help doctors arrive at a diagnosis. Common tics are not often misdiagnosed by knowledgeable clinicians. However, atypical symptoms or atypical presentations (for example, onset of symptoms in adulthood) may require specific specialty 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)
- computerized tomography (CT)
- electroencephalogram (EEG) studies
- certain blood tests
may be used to rule out other conditions that might be confused with TS when the history or clinical examination is atypical.
It is not uncommon for patients to obtain a formal diagnosis of TS only after symptoms have been present for some time. The reasons for this are many. For families and physicians unfamiliar with TS, mild and even moderate tic symptoms may be considered inconsequential, part of a developmental phase, or the result of another condition. For example, parents may think that eye blinking is related to vision problems or that sniffing is related to seasonal allergies. Many patients are self-diagnosed after they, their parents, other relatives, or friends read or hear about TS from others.
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 be placed in an educational setting that meets their individual needs. Students may require:
- smaller or special classes
- in some cases special schools.
All students with TS need a tolerant and compassionate setting that both encourages them to work to their full potential and is flexible enough to accommodate their special needs. This setting may include a private study area, exams outside the regular classroom, or even oral exams when the child's symptoms interfere with his or her ability to write. Untimed testing reduces stress for students with TS.
Neuroleptics (drugs that may be used to treat psychotic and non-psychotic disorders) are the most consistently useful medications for tic suppression; a number are available but some are more effective than others:
Unfortunately, there is no one medication that is helpful to all people with TS, nor does any medication completely eliminate symptoms. In addition, all medications have side effects. Many neuroleptic side effects can be managed by initiating treatment slowly and reducing the dose when side effects occur. The most common side effects of neuroleptics include
- weight gain
- cognitive dulling
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. One form of dyskinesia called tardive dyskinesia is a movement disorder distinct from TS that may result from the chronic use of neuroleptics. The risk of this side effect can be reduced by using lower doses of neuroleptics for shorter periods of time.
Other medications may also be useful for reducing tic severity, but most have not been as extensively studied or shown to be as consistently useful as neuroleptics. Additional medications with demonstrated efficacy include alpha-adrenergic agonists such as clonidine and guanfacine. These medications are used primarily for hypertension but are also used in the treatment of tics. The most common side effect from these medications that precludes their use is sedation. However, given the lower side effect risk associated with these medications, they are often used as first-line agents before proceeding to treatment with neuroleptics. Effective medications are also available to treat some of the associated neurobehavioral disorders that can occur in patients with TS. Recent research shows that stimulant medications such as methylphenidate and dextroamphetamine can lessen ADHD symptoms in people with TS without causing tics to become more severe. However, the product labeling for stimulants currently contraindicates the use of these drugs in children with tics/TS and those with a family history of tics. Scientists hope that future studies will include a thorough discussion of the risks and benefits of stimulants in those with TS or a family history of TS and will clarify this issue.
For obsessive-compulsive symptoms that significantly disrupt daily functioning, the serotonin reuptake inhibitors:
have been proven effective in some patients.
Behavioral treatments such as awareness training and competing response training can also be used to reduce tics. A recent NIH-funded, multi-center randomized control trial called Cognitive Behavioral Intervention for Tics, or 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 sometimes occur.
Family and Community Resources
P.O. Box 5801
Bethesda, MD 20824
Current Medical Research
Knowledge about TS comes from studies across a number of medical and scientific disciplines, including:
- clinical trials (medication and non-medication)
- descriptive/diagnostic clinical science.
- Genetic studies. Currently, NIH-funded investigators are conducting a variety of large-scale genetic studies. Rapid advances in the technology of gene discovery will allow for genome-wide screening approaches in TS, and finding a gene or genes for TS would be a major step toward understanding genetic risk factors. In addition, understanding the genetics of TS genes may strengthen clinical diagnosis, improve genetic counseling, lead to the clarification of pathophysiology, and provide clues for more effective therapies.
- Neuroimaging studies. Advances in imaging technology and an increase in trained investigators 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.
- Neuropathology. There has been an increase in the number and quality of donated postmortem brains from TS patients available for research purposes. 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. A number of clinical trials in TS have recently been completed or are currently 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.
- Epidemiology and clinical science. Careful epidemiological studies now estimate the prevalence of TS to be substantially higher than previously thought with a wider range of clinical severity. Furthermore, 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, a new appreciation of sensory tics, and the role of co-existing disorders in rage attacks. 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 a number of epidemiological and clinical investigations currently underway in this intriguing area.
Famous People Who Have Tourette Syndrome
Goggle's Website. (2015). Tourette Syndrome Images. Web. Retrieved from https://www.google.com/search
NINDS. (2012). Tourette Syndrome Fact Sheet. National Institute of Neurological Disorders and Stroke. NIH Publication No. 12-2163. Web. Retrieved from http://www.ninds.nih.gov/disorders/tourette/detai...
WebMD. (2014). What Is Tourette Sydrome? WebMD Medical Reference. Web. Retrieved from http://www.webmd.com/brain/tourettes-syndrome