What is Tourette syndrome?

Tourette syndrome is a tic disorder that starts in childhood. The prime symptom is to have repeated tics. A tic is a sudden movement or sound that is repeated over and over. A tic has no purpose and, in general, you cannot help doing it. For example, repeated blinking, repeated throat clearing, repeated head nodding, etc. Tics are very common in children and usually last less than one year. However, children with Tourette syndrome have many types of tics of sudden movements and noises and the tics persist for more than a year.

The syndrome is named after the person who described the condition in 1885, Dr Georges Gilles de la Tourette. It is also known by the full name of Gilles de la Tourette('s) syndrome.

The main symptom is many (multiple) tics. These are classified as either movement tics (motor tics) or vocal tics.

• Motor tics include movements such as blinking, head turning, head nodding, kicking, mouth pouting, mouth opening, mouth twitches, etc.

• Vocal tics include things such as throat clearing, coughing, sniffing, yelling, or making animal sounds.

Tics occur very commonly in children who do not have Tourette syndrome. In fact, up to 1 in 5 children will develop a tic at some stage. These tics are often minor and of little significance, usually come and go and often disappear within a year.

In Tourette syndrome, a child develops multiple tics which occur many times a day (often in bouts) and the tics last for more than one year. The child has a combination of motor and vocal tics. The tics cause some degree of difficulty in school or socially. The tics tend to come and go and sometimes cause more trouble than at other times. They may be worse when the child is anxious or stressed. They may be better when the child is concentrating on something else, such as an activity or task. Trying to prevent the tic causes a feeling of stress.

There are several other symptoms that sometimes occur. These include:

• Coprolalia - the involuntary use of obscenities and swear words. This is perhaps the most famous symptom which the media tend to focus in on. However, this only occurs in about 1 in 10 children with Tourette syndrome. Nota: it must be emphasised that if this occurs, the child cannot help swearing. It is not a reflection on their moral character or upbringing.

• Echophenomena - copying what others say and do.

• Palilalia - repeating your own words.

• Non-obscene socially inappropriate (NOSI) behaviours - such as making inappropriate or rude personal comments.

The cause is not known. There are various theories which include the following:

Genetic. It is generally believed that abnormalities in genes are responsible for most cases of Tourette syndrome. Genes are passed on to a child from each parent and determine what we look like, how our body functions and even what diseases we may get. A child is more likely to develop Tourette syndrome if they have a father, mother, brother or sister with it.

Neurológico. Some studies have shown that there are some minor defects in the structure and working of the brain in children with Tourette syndrome. Studies also suggest that there may be a problem with one of the brain's chemicals called dopamine. There is not a lot of information available about the detail or significance of these changes.

Environmental. Although it has not been proven, there is some evidence that problems during pregnancy or childbirth may increase the risk of a child developing Tourette syndrome. This may include problems such as prolonged labour, high levels of maternal stress in pregnancy, or babies with very low birth weight. There is also possibly a connection with a particular infection with the streptococcal germ in some children with Tourette syndrome. This is a fairly recent discovery and not very much is known about the link.

Tourette syndrome is much more common than previously thought. Up to 1 in 100 children have the condition, although it varies in how mild or severe it is. It is about four times more common in boys than in girls. Tourette syndrome is equally common in all racial and ethnic groups and all socio-economic backgrounds. The condition begins between 2-14 years of age (the average age is 6).

If a child has Tourette syndrome they are also likely to have one or more related conditions. It is not clear why these other conditions occur at the same time. Only about 1 child in 10 with Tourette syndrome has no other associated problem. The most common conditions seen with Tourette syndrome are listed below with how often they occur in children with Tourette syndrome.

• Transtorno obsessivo-compulsivo (TOC), or obsessive-compulsive behaviour (OCB). This occurs in about 6 in 10 children with Tourette syndrome.

• Attention deficit hyperactivity disorder (ADHD, or ADD). This occurs in about 6 in 10 children with Tourette syndrome.

• Learning difficulties. These occur in about 3 in 10 children with Tourette syndrome.

• Mood disorders such as depression or anxiety occur in about 2 in 10 children with Tourette syndrome.

• Conduct disorders. These occur in about 1-2 in 10 children with Tourette syndrome.

• Self-harming behaviours such as head banging occur in about 3 in 10 children with Tourette syndrome.

• Behavioural problems. Up to 8 in 10 children with Tourette syndrome will also have some degree of behavioural problems.

There is no blood test or particular investigation for Tourette syndrome. The diagnosis is based on the symptoms and is made after a careful discussion with you and your child as well as a physical examination to rule out other disorders. This will usually be done by a doctor with a special interest in the brain and nervous system (a neurologist). Some tests may be done to rule out other conditions, such as neurological conditions, which could account for the tics.

People with Tourette syndrome are referred to a specialist for advice about the best treatment. Particularly if there are other associated conditions, choosing the right treatment can be tricky and needs an expert. For example, some treatments for ADHD can make tics worse.

• Talking/behavioural treatments. Various talking (psychological) treatments can help to ease the symptoms and problems of Tourette syndrome.

  • These include cognitive behavioural therapy (CBT).

  • Habit reversal training.

  • Exposure and response prevention therapy.

These treatments aim to help the child to recognise and control their tics. Often children are able to learn to suppress the tics during the day at school but will need an outlet to release them at home.

• Education and support. There is some evidence that providing parents and children with Tourette syndrome with information about the condition, results in better outcomes.

  • Grupos de apoio. You may find contact with one of the support groups such those listed in Further reading below helpful, as they can provide further information on the condition and can even put you in contact with others in the same situation.

  • Support from caregivers. It is very important that your child's schoolteacher and others who are involved in your child's care should be informed and educated about the best way to manage this condition. Your child's teacher can have a very positive impact on your child's behaviour and development if they understand the problems that may be present.

  • Individual approach. In addition to any usual treatments, individual children may respond to different approaches. Parents and teachers can build on whatever works. For example, some children can gain more control of their lives through the medium of dance.

• Medicação. In more severe Tourette syndrome, medicines can sometimes help to reduce the occurrence of tics.

  • The medicines that are mostly used are from the group known as antipsicóticos: mostrou que não preveniu a condição, mas esteve associado a uma duração mais curta e a uma permanência hospitalar menor, risperidona, aripiprazole e sulpiride.

  • Medicines such as clonazepam - a benzodiazepine - have been used in the past but are usually avoided as they are addictive.

  • Newer medicines such as topiramate - used for migraine and epilepsy - are being studied for Tourette syndrome. The aim of treatment with medicines is to control the tics to a point that will enable your child to function well while minimising side-effects. The tics may not disappear completely.

• Management of other conditions. If your child has other conditions present with Tourette syndrome then it is important that these should also be treated and managed well.

• Other treatments. The use of surgery in Tourette syndrome is only recommended for people with severe symptoms who do not respond to other treatment.

  • Deep brain stimulation using electrodes implanted into the brain has produced encouraging results in limited trials. However, more research is needed to see whether it is safe to be used more generally.

  • Botulinum toxin (Botox®) injections are sometimes used for individual tics, but need to be repeated regularly.

Many children with Tourette syndrome improve over time. By the time they are adults, in many cases the symptoms have eased considerably or have gone. However, some children with this condition will continue to have marked symptoms into adulthood and, although the tics tend to stabilise over time, some new tics may develop.