Tourette syndrome

   

Tourette syndrome — also called Tourette's syndrome, Tourette Spectrum (TS), Tourette's disorder, or Gilles de la Tourette syndrome (after its discoverer, Georges Gilles de la Tourette) — is a neurological or neurochemical disorder characterized by tics — involuntary, rapid, sudden movements or vocalizations that occur repeatedly in the same way. Symptoms include multiple motor and one or more vocal tics present at some time during the disorder although not necessarily simultaneously; the occurrence of tics many times a day (usually in bouts) nearly every day or intermittently throughout a span of more than one year; the periodic change in the number, frequency, type and location of the tics, and in the waxing and waning of their severity; symptoms disappearing for weeks or months at a time; and onset before the age of 18. Vocal tics may fall into various categories, including echolalia (the urge to repeat words spoken by someone else after being heard by the person with the disorder), palilalia (the urge to repeat one's own previously spoken words), lexilalia (the urge to repeat words after reading them) and, most controversially, coprolalia (the spontaneous utterance of socially objectionable words, such as obscenities and racial or ethnic slurs). There are many other vocal tics besides those categorized by word repetition - in fact, a TS tic can be almost any possible short vocal sound, with the most common tics resembling throat clearing, short coughs, grunts, or moans.

The term "involuntary" used to describe TS tics is a source of confusion since it is known that most people with TS do have some control over the symptoms. Before tic onset, individuals with TS experience what is called a "premonitory urge," similar to the feeling of an itch. What is recognized is that the control which can be exerted from seconds to hours at a time may merely postpone more severe outbursts of symptoms. Tics are experienced as irresistible as an itch and must eventually be expressed. People with TS often seek a secluded spot to release their symptoms after delaying them in school or at work. Typically, tics increase as a result of tension or stress (but are not caused by stress) and decrease with relaxation or concentration on an absorbing task. In fact, neurologist and writer Oliver Sacks has described a man with severe TS who is both a pilot and a surgeon.

Other commonly associated symptoms are obsessive compulsive disorder (OCD) and attention deficit hyperactivity disorder (ADHD).

TS affects up to 1.13% of children with a wide range of severity, but can in some cases decrease in severity after adolescence, or in rare occurences show an almost complete cessation of tics in some adults with TS. The severity of tics can range vastly, from one or two almost imperceptible tics such as repetitive blinking or twitching fingers, which barely affect the sufferer's quality of life, to almost constant full body spasms with loud vocal tics and shouted coprolalia, which can inhibit or prevent the TS sufferer from functioning normally in society and engaging in common activites such as holding a job, having an fulfilling social life, or maintaining his/her basic needs. This is compounded further by the stigma attached to TS and the notable lack of social acceptance and understanding surrounding the disorder. The entertainment industry has often depicted Tourette's sufferers in movies and television (for example, the movie "Deuce Bigalow: Male Gigolo") as being social misfits whose only tic is coprolalia (apparently considered the "humorous" symptom of TS, since in comparison to physical or non-word repetition vocal tics, coprolalia is a relatively rare symptom), which has led to the general public's understanding of Tourette's sufferers to be limited to "people who can't help yelling swear words a lot". Obviously this misconception can lead to many TS sufferers having a difficult time integrating into society, whether or not they themselves exhibit coprolalia as a symptom.

(Case in point: A review of the Edit History for this Wikipedia article shows a considerable number of anonymous edits in which people randomly insert swear words into the article, presumably to imply that this would be how a TS sufferer would read the article aloud.)


Treatment

The majority of people with TS require no medication, but medication is available to help when symptoms interfere with functioning. TS medications are only able to help reduce specific symptoms. Neuroleptic and antihypertensive drugs can have long- and short-term side effects, and use of stimulants is controversial. Relaxation techniques and biofeedback may be useful in alleviating stress.

Until 2004, there wasn't a cure for TS. However, doctors in the US successfully carried out a brain surgery in February 2004, in which tiny electrodes powered by batteries inserted in the chest, were placed beside the thalamus in each cerebral hemisphere. Within half a minute of activating the electrodes, the patient could walk normally and displayed a complete lack of symptoms. However, this is a dangerous procedure and is unlikely to become widespread. In any case, the condition in many individuals improves as they mature. Individuals with TS can expect to live a normal life span. Although TS is generally lifelong and chronic, it is not degenerative. In a few cases, complete remission occurs after adolescence, and in many cases total remission occurs after the age of 40.

Cognitive Behavioural Therapy (CBT) can be used to try to disrupt the automatic chain of events underlying the tics.

Genetics

Genetic studies indicate tic disorders, including TS, are inherited as a dominant gene(s) that may produce varying symptoms in different family members. A person with TS has about a 50% chance of passing the gene(s) to one of his/her children. However, the gene(s) may express as TS, as a milder tic disorder, or as obsessive compulsive symptoms with no tics at all. It is known that a higher than usual incidence of milder tic disorders and obsessive compulsive behaviours are more common in the families of TS patients. The sex of the child also influences the expression of the gene(s). The chance that the child of a person with TS will have the disorder is at least three times higher for a son than for a daughter. Yet only a minority of the children who inherit the gene(s) will have symptoms severe enough to ever require medical attention. In some cases, TS may not be inherited; these cases are identified as "sporadic" TS because a genetic link cannot be found.

Neurology

The basal ganglia have been implicated in Tourette's. It is hypothesised that, in Tourette's, groups of neurons within the basal ganglia fail to inhibit unwanted behaviours and so these behaviours bubble up as tics. The thalamus forms part of the circuit connecting the basal ganglia and the cerebral cortex.

Dopamine blockers can alleviate the symptoms of Tourette's.

There is a theory that streptococcal infection (the cause of rheumatic fever) might cause some cases of Tourette's. Some children show their first signs of Tourette's after a streptococcal infection. Immonological studies have suggested that in some children the antibodies generated to combat strep misidentify and damage neurons in the basal ganglia.

Alternative medicine

Alternative medicine tends to hold the view that TS is caused by yeast infections and mercury poisoning (see gluten-free, casein-free diet).

People with Tourette's

References

External Links



nl:Syndroom van Gilles de la Tourette

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