TOURETTE SYNDROME

Tourette syndrome is an inherited neurological disorder with onset in childhood, characterized by the presence of multiple physical (motor) tics and at least one vocal tic; these tics characteristically wax and wane. It is a co-morbid disorder with Autism Spectrum Disorders such as autism and Aspergers syndrome.

Tourette’s is defined as part of a spectrum of tic disorders, which includes transient and chronic tics. It is also called Tourette’s syndrome, Tourette’s disorder, Gilles de la Tourette syndrome, GTS or the more common Tourette’s or TS.

Tourette’s was once considered a rare and bizarre syndrome, most often associated with the exclamation of obscene words or socially inappropriate and derogatory remarks (coprolalia). However, this symptom is present in only a small minority of people with Tourette’s. Tourette’s is no longer considered a rare condition, but it may not always be correctly identified because most cases are classified as mild cases.

Since the incidence may be as high as one in a hundred people, up to 530,000 U.S. school-age children may have Tourette’s, with the more common tics of eye blinking, coughing, throat clearing, sniffing, and facial movements. People with Tourette’s have normal life expectancy and intelligence. The severity of the tics decreases for most children as they pass through adolescence, and extreme Tourette’s in adulthood is a rarity. Notable individuals with Tourette’s are found in all walks of life.

Genetic and environmental factors each play a role in the etiology of Tourette’s, but the exact causes are unknown. In most cases, medication is unnecessary. There is no effective medication for every case of tics, but there are medications and therapies that can help when their use is warranted. Explanation and reassurance alone are often sufficient treatment; education is an important part of any treatment plan.

Characteristics of Tourette syndrome

Tics are sudden, repetitive, stereotyped, non-rhythmic, involuntary movements and utterances that involve discrete muscle groups. Motor tics are movement-based tics, while vocal tics are involuntary sounds produced by moving air through the nose, mouth, or throat.

The most typical age of onset is from five to seven. The ages of highest tic severity are eight to twelve (average ten), with tics steadily declining for most patients as they pass through adolescence. The most common, first-presenting tics are eye blinking, facial movements, sniffing and throat clearing. Initial tics present most frequently in midline body regions where there are many muscles, usually the head, neck and facial region. This can be contrasted with the stereotyped movements of other disorders (such as stims and stereotypes of the Autism Spectrum Disorders), which typically have an earlier age of onset, are more symmetrical, rhythmical and bilateral, and involve the extremities (e.g., flapping the hands). Tics that appear early in the course of the condition are frequently confused with other conditions, such as allergies, asthma, and vision problems: pediatricians, allergists and ophthalmologists are typically the first to see a child with tics.

Unlike tics in other disorders, the tics of Tourette’s are temporarily suppressible and preceded by a premonitory urge. Immediately preceding tic onset, most individuals with Tourette’s are aware of an urge that is similar to the need to sneeze or scratch an itch. Individuals describe the need to tic as a buildup of tension which they consciously choose to release, as if they “had to do it”. Children may be less aware of the premonitory urge associated with tics than are adults, but their awareness tends to increase with maturity.

Causes of Tourette syndrome

The exact cause of Tourette’s is unknown, but it is well established that both genetic and environmental factors are involved. Genetic studies have proved that the overwhelming majority of cases of Tourette’s are inherited, although the exact mode of inheritance is not yet known, and no gene has been identified.

Non-genetic, environmental, infectious, or psychosocial factors — while not causing Tourette’s — can influence its severity. Autoimmune processes may affect tic onset and exacerbation in some cases. The unproven and contentious hypothesis that Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections plays a role in the onset of tic disorders and OCD is a current focus of research.

Diagnosis of Tourette syndrome

According to the fourth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-TR), Tourette’s Disorder may be diagnosed when a person exhibits both multiple motor and one or more vocal tics (although these do not need to be concurrent) over the period of a year, with no more than three consecutive tic-free months. Previous editions of the DSM included a requirement for “marked distress or significant impairment in social, occupational or other important areas of functioning”, but this requirement was removed in the most recent update of the manual, in recognition that clinicians see patients who meet all the other criterion for Tourette’s, but do not have distress or impairment.

Treatment of Tourette syndrome

The treatment of Tourette’s focuses on identifying and helping the individual manage the most troubling or impairing symptoms. Most cases of Tourette’s are mild, and do not require pharmacological treatment; instead, psychobehavioral therapy, education, and reassurance may be sufficient. Relaxation techniques, such as exercise, yoga or meditation, may be useful in relieving the stress that may aggravate tics, but the majority of behavioral interventions (such as relaxation training and biofeedback, with the exception of habit reversal) have not been systematically evaluated and are not empirically supported therapies for Tourette’s. Educating a patient, family, and surrounding community (such as friends, school, and church) is a key treatment strategy.

Treatments, where warranted, can be divided into those that target tics and comorbid conditions, which, when present, are often a larger source of impairment than the tics themselves. Not all people with tics have comorbid conditions, but when those conditions are present, such as Aspergers syndrome, they often take treatment priority.

There is no cure for Tourette’s and no medication that works universally for all individuals without significant adverse effects. Clonidine (or the clonidine patch) is one of the medications typically tried first when medication is needed for Tourette’s. Haloperidol is an antipsychotic medication sometimes used to treat severe cases of Tourettes . Medication is available to help when symptoms interfere with functioning.

The classes of medication with the most proven efficacy in treating tics include typical and atypical neuroleptics, antihypertensive agents and stimulants may be useful in treating ADHD when it co-occurs with tic disorders.

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