TOURETTE SYNDROME

What is it?

Tourette Syndrome (TS) is a neurological disorder characterised by tics (involuntary, rapid, sudden movements that occur repeatedly in the same way).

Who gets it?

The cause has not been established, although current research presents considerable evidence that the disorder stems from the abnormal metabolism of at least one brain chemical (neurotransmitter) called dopamine. Undoubtedly, other neurotransmitters (e.g. serotonin) are also involved.

Genetic studies indicate that TS is inherited as a dominant gene that causes varying symptoms in different family members. A person with TS has about a 50% chance of passing on the gene to one of his/her children. However, that genetic predisposition may express itself as TS, as a milder tic disorder, or as obsessive compulsive symptoms with no tics at all. It is known that a higher than normal incidence of milder tic disorders and obsessive compulsive behaviours occur in the families of TS patients.

The sex of the child also influences the expression of the gene. The chance that the child of a person with TS will have symptoms is at least three to four times higher for a son than for a daughter. Yet only about 10% of the children who inherit the gene have symptoms severe enough to ever require medical attention. In some cases TS may not be inherited, and is identified as sporadic TS. The cause in these cases is unknown.

What are the symptoms?

The symptoms include:

• both multiple motor and one or more vocal tics present at some time during the illness, 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
• periodic change in the number, frequency, type and location of the tics, and in the waxing and waning of their severity; symptoms can sometimes disappear for weeks or months at a time
• onset before the age of 21

There is no such thing as a typical TS case. The expression of symptoms covers a spectrum from very mild, which is true of most people, to quite severe.

The term "involuntary", used to describe TS tics, is sometimes confusing since it is known that most people with TS do have some control over their symptoms. What is not recognised 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 and eventually must 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, and decrease with relaxation or concentration on an absorbing task.

Not all TS sufferers exhibit other associated behaviour, but many do have additional problems.

Obsessive compulsive and ritualistic behaviour occurs when the person feels that something must be done repeatedly. Examples include touching an object with one hand after touching it with the other hand to "even things up", or repeatedly checking to see that the heat on the oven is turned off. Children sometimes beg their parents to repeat a sentence many times until it "sounds right".

Hyperactivity with or without Attention Deficit Disorder (ADHD) occurs in many people with TS. Children may show signs of hyperactivity before TS symptoms appear. Indications of ADHD may include: difficulty with concentration; failing to finish what is started; not listening; being easily distracted; often acting before thinking; shifting constantly from one activity to another; needing a great deal of supervision; and general fidgeting. Adults may also exhibit signs of ADHD, such as overly impulsive behaviour and concentration difficulties.

Learning disabilities such as reading and writing difficulties, arithmetic disorders, and perceptual problems, may occur. Difficulties with impulse control may result (in rare instances) in overly aggressive behaviour or socially inappropriate acts. Also, defiant and angry behaviours can occur. Sleep disorders are fairly common among people with TS. These include frequent awakenings or walking or talking in one's sleep.

What tests will the Doctor want to do?

The diagnosis is made by observing the symptoms and by evaluating the history of their onset. No blood analysis or other type of neurological testing exists to diagnose TS. However, some physicians may wish to order an EEG, MRI, CAT scan, or certain blood tests to rule out other ailments that might be confused with TS. Rating scales are available for assessment of tic severity.

What is the treatment?

There is no known cure at present. However, some people experience marked improvement in their late teens or early twenties. Many of the people with TS get better, not worse, as they mature, and those diagnosed with TS can anticipate a normal life span. There are several reports of a complete remission of symptoms.

The majority of people with TS are bled by their tics or behavioural symptoms, and their medication. However, some medications interfere with function. Lude haloperidol (Haidol), clonidine (Catapres), pi'mi nazine (Prolixin, Permitil), and clonazepam (Kionopji as Ritalin), Cylert, and Dexedrine, that are prescribed for hyperactivity may increase tics. Their use is controversial. For obsessive compulsive traits which interfere significantly with daily functioning, fiuoxedne (Prozac) and clomipramine (Anafranil) are prescribed.

The dosage necessary to achieve maximum control of symptoms varies for each patient and must be gauged carefully by a doctor. The medicine is administered in small doses with gradual increases to the point where there is a maximum alleviation of symptoms, with minimal side-effects. Some of the undesirable reactions to medications are weight gain, muscular rigidity, fatigue, and motor restlessness, most of which can be reduced with specific medications. Side-effects that include depression and cognitive impairment can be alleviated with dosage reduction or a change of medication.

Other types of therapy may also be helpful. Psychotherapy can assist a person with TS and help the family cope, and some behaviour therapies can teach the substitution of one tic for another that is more acceptable. The use of relaxation techniques and/or biofeedback can serve to alleviate stress reactions that cause tics to increase.

It is important to treat TS early, especially in those instances when the symptomatology of the condition is viewed by some people as bizarre, disruptive, and frightening. TS symptoms frequently provoke ridicule and rejection by peers, neighbours, teachers, and even casual observers. Parents may be overwhelmed by the strangeness of their child's behaviour. The child may be threatened, excluded from activities, and prevented from enjoying normal interpersonal relationships. These difficulties may become greater during adolescence, an especially trying period for young people and even more so for a person coping with a neurological problem. To avoid psychological harm, early diagnosis and treatment are crucial. Moreover, in more serious cases, it is possible to control the symptoms with medication.