Tourette syndrome is an inherited neurological disorder. Symptoms, which begin in childhood are tics (various, physical or motor) and at least one phonic tic or vocal tic. Tics can become severe or be mild and can fluctuate in occurrence. Tourette syndrome is defined in as a spectrum disorder in which the tics can be chronic or transient.
Tourette syndrome has also been referred to as GTS, Tourette’s, TS Tourette’s syndrome, and Gilles de la Tourette syndrome. At one time it was considered rare and quite unusual. Most often it is associated with verbal outburst of foul or derogatory language known as coprolalia. This behavior actually only occurs in a small portion of patients with Tourette’s syndrome.
The name Tourette syndrome comes from Georges Albert Edouard Brutus Gilles de la Tourette. He was a medical resident under the tutelage of Jean-Martin Charcot. Other famous students of Charcot were Sigmund Freud and Alfred Binet, who designed the first IQ test.
Tourette’s syndrome is one of many tic disorders which are classified by duration and type. The duration of tics is classified as chronic or transient. Chronic tics are either a single motor tic or a phonic tic, but not both that occur for more than a year. Transient tics consist of multiple motor tics, phonic tics or a combination of the two. Transient tics last from four weeks until a year.
Tics are phonic (vocal sounds) or motor (physical movements) which occur spontaneously and without cause. A tic is not a reflex. Tics occur while normal physical and vocal behavior is occurring without the control of the individual. Tics that are common in Tourette’s syndrome change in the frequency of occurrence, variety, location and severity.
Coprolalia is the spontaneous verbal outburst of objectionable language, words or phrases. It occurs in fewer than 15% of diagnosed patients. Palilalia is the repetition of words and occur in a few cases. The most common tics are eye blinking and throat clearing.
Tourette’s disorder tics can be suppressed sometimes, because a patient can learn the ‘premonitory urge’ that occurs before the tic.
Tics are defined as semi-voluntary or unvoluntary. Tics are suppressible and irresistible. Patients with Tourette’s can suppress tics for a limited time period, but eventually may have an ‘outburst’ of tics. Tourette’s patients sometimes seek a secluded spot to release their tics or may have a increased presentation of tics after work or school (environments in which a prolonged attempt at control has occurred).
Patients may struggle to determine the premonitory urge, especially children. The ability to suppress tics is usually more developed in adults than children.
A definitive cause for Tourette’s syndrome has not been discovered. Genetic and environmental factors have been determined to be involved in the development of Tourette’s syndrome.
Patients with Tourette’s have a 50% chance of having a child with Tourette’s or a form of Tourette’s. The symptoms may or may not be the identical in severity or form. Males are more likely to develop Tourette’s syndrome than females.
Environmental, infectious and psychosocial factors can agitate symptoms and increase the severity of Tourette’s syndrome. Tics are believed to be caused by a dysfunction in the subcortical and cortical regions of the brain. Imaging of the brain have detected failures in the circuits of the basal ganglia and the frontal cortex.
Tourette’s is diagnosed based on the criteria outlined in the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-TR). Tourette’s syndrome is diagnosed when a person presents with multiple motor tics and at least one or more vocal tics over twelve month’s time, with no more than three months of experiencing no tics. The tics begin before the age of eighteen and can not be attributed to other physiological aspects. Other conditions such as Autism must be ruled out.
There are no laboratory screenings for Tourette’s syndrome. The diagnosis is made completely on observation, family history and eliminating any secondary causes of other tic disorders. Seizures should be ruled out through a EEG. Brain abnormalities should be ruled out using medical imaging. Hypothyroidism can cause tics and can be detected through a TSH blood test. A urine drug screening should be performed on older teenagers and young adults if the tics appear suddenly. This could be a sign of cocaine or stimulant use. A family history of liver disease should be noted and the patient should have a screening for Wilson’s disease. The screening will check for serum copper and ceruloplasmin levels.
Secondary causes of tics, referred to as tourettism, need to be ruled out. Conditions that may cause tics are autism spectrum disorders, Duchenne muslcular dystrophy, Hallervorden-Spatz syndrome, idiopathic dystonia, Wilson’s disease, Sydenham’s chorea, tuberous scleroisis, Fragile-X syndrome, Klinefelter’s syndrome, Down Syndrome, and Huntington’s disease.
Acquired causes of tics are head trauma, drug abuse, encephalitis, stroke and CO
There is no cure for Tourette’s syndrome. Treatment is aimed at managing symptoms to improve quality of life. Treatment is decided on a case by case basis, with the individual determining what is negatively impacting their life.
Treatment can include behavior modification and medication. Clonidine is a medication that is usually tried first for patients with Tourette’s syndrome.
Cognitive behavioral therapy can be used to treat Tourette’s patients. This therapy is designed to produce a habit reversal of tics.
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