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Word On Tourettes

Our grateful thanks to Suzanne Dobson from the charity Tourettes Action for her contribution to our on-air report, which you can hear via the radio player further down this page). Thank you also to the charity for the use of the information below. To find more about Tourette Syndrome and Tourettes Action click here.

What is Tourette Syndrome (TS)? It is an inherited, neurological condition, the key features of which are tics, involuntary and uncontrollable sounds and movements. TS is sometimes known as Multiple Tic Disorder or Tic Spectrum Disorder.

TS is a complex condition and covers a wide spectrum of symptoms. People may have a mild form, and they and those close to them may not even be aware that they have TS. At the other end of the scale, medical symptoms are extreme and the social, educational and economic effects can be serious. These are the examples that generate media interest and generate some of the stereotypes that exist about TS.

Destroying the myths about TS  One of the most common beliefs is that all people with TS swear uncontrollably. However, only 10 percent of people with TS have a swearing tic, which is called coprolalia (see below). Each person with TS has different tics and will experience diverse symptoms. For people with more severe TS the impact on their quality of life is significant.

  • Physically - tics can cause damage to joints or self-injury, eg hitting oneself
  • Educationally - TS can make it impossible to follow what is going on in class
  • Economically - TS can be a barrier to employment
  • Socially - TS can lead to ridicule, bullying, and social exclusion

In many cases TS is linked to other behaviors, most often Obsessive Compulsive Disorder (OCD) and Attention Deficit Disorder (ADD). For these reasons treatment of TS is very particular to each individual and should be discussed with a TS specialist.

Who has TS?  Because many people with mild TS are not affected to the extent that the condition has a significant impact on their quality of life, not everybody with TS will be diagnosed as they have no need to access treatment. This makes it difficult to know for certain how many people are living with the condition. However, it is estimated that TS affects one schoolchild in every hundred and more than 300,000 children and adults in the UK live with the condition.

TS is a neurological condition. It also has a genetic link, so there will probably be someone in your wider family who also has TS, although they may have a mild form of the condition and might not have realized they have it, especially if they had the condition in childhood but not today.

TS typically starts in childhood and the average age for diagnosis in the UK is seven years. For about half of children with TS the condition will continue into adulthood. The other 50 percent will see a reduction in the symptoms of their TS by the end of their teenage years.

Symptoms of TS  The key features are tics; both repeated movements and sounds that are chronic (long-term) and involuntary. Someone with TS may be able to suppress their tics for a period but eventually, they have to let them out.

Tics usually start in childhood around the age of seven and are usually worst between 10-12 years. However, in approximately half of the people with TS, most symptoms disappear by the age of 18. Each person with TS will have different symptoms. Some people may have a mild form, and they and those close to them may not even be aware that they have TS.

Over 85 percent of people with TS have more than just tics. Additional conditions (comorbidities) include obsessive-compulsive disorder (OCD) as well as attention deficit hyperactivity disorder (ADHD). Children and adults may also suffer from ‘rages’. Comorbidities often cause more problems than the tics and can be less visible.

Types of TS  Generally, TS can be classified corresponding to levels of severity and complexity and whether other conditions can occur together with TS.

Pure TS – motor (movement) and vocal tics only.

Full blown TS - Paliphenomena, echophenomena, copropraxia/coprolalia, NOSI, movement and sound tics. 

TS plus - OCB or OCD, ADHD, depression, anxiety, SIB, sleep disorders, personality disorders, other psychopathology, conduct disorder, paliphenomena, echophenomena, copropraxia/coprolalia, NOSI, movement and sound tics.*

(As Tourettes Action remind us - that this information is a guide only. Tics and behaviors vary widely, and it is possible, for example, to have OCB and sleep disorders without coprolalia.)

Glossary

  • Copropraxia - the making of obscene or otherwise unacceptable movements or gestures. 
  • Coprolalia - using obscene or unacceptable language.
  • Coprophenomena - the involuntary expression of socially unacceptable words or gestures.
  • Echophenomena - repeating other people’s words (echolalia) and other people’s gestures (echopraxia).
  • NOSI - Non-Obscene Socially Inappropriate behavior, involves saying things that are socially unacceptable.
  • Paliphenomena - Similar to echophenomena. Involves the person with TS repeating their own words and actions such as "Hello, I came here by bus bus bus bus".

Types of tics  Tics often start around the head and face, like blinking and/or grimacing, these are known as motor tics; vocal tics tend to appear later. The different tics can be simple, such as blinking, or complex, like jumping.

Motor tics  Simple: Eye blinking, head jerking, shoulder shrugging and facial grimacing.

Complex: Jumping, touching other people or things, smelling, twirling and sometimes hitting or biting oneself.

Sound

Simple  Throat clearing, yelping, and other noises sniffing, coughing and tongue clicking.

Complex Uttering words or phrases out of context, coprolalia (saying socially unacceptable words), and echolalia (repeating a sound, word, or phrase just heard).

Even within the same person, tics vary in many ways:

  • they wax and wane; get better and worse over time
  • they change; one tic stops and another starts
  • they may be made worse by stress and anxiety
  • they may be alleviated with relaxation or concentration on an absorbing task

In some situations, it may be difficult to believe these noises or actions that people with TS make are involuntary, but they are part of the symptoms of TS.

TS and swearing - coprolalia  As we highlighted above it is commonly assumed that everybody with TS swears. However, only 10 percent of people with TS have a swearing tic, known as coprolalia. Some people also have copropraxia, making rude gestures.

Non-Obscene Socially Inappropriate (NOSI) behavior affects some people with TS, where they have a compulsion to do or say the wrong thing, which may be socially unacceptable; for example, touching something they should not, or commenting on somebody’s physical appearance.

Some people with TS have vocal tics that take the form of random words or phrases or repeating what you or someone else has just said echolalia.

Suppressing tics  Although the word ‘involuntary’ is generally used to describe the movements and sounds, most people with TS can suppress their tics for a short time. This could be compared to the experience of suppressing a sneeze. A good way to experience this is to see how long you can avoid blinking for. The longer you hold your eyes open, the greater the urge to blink. This is similar to how it feels to need to tic.

Suppressing tics does not mean they go away, instead, the tics are deferred and can be stronger when they are finally released. Some people with TS refer to this as a tic attack. When possible, it is best to let out tics as and when they come.

It is important, however, to remember that tics are involuntary and that people cannot help but let them out. Tics are not a behavioral issue.

Tic disorders or Tourette Syndrome?  Some people may be diagnosed with a tic disorder rather than Tourette Syndrome. The following is a brief description of the various tic disorders.

Transient tic disorder - consists of multiple motor and/or phonic tics which last at least 4 weeks, but less than 12 months

Chronic tic disorder - either single or multiple motor or phonic tics, but not both, which are present for more than a year

Tourette Syndrome (TS) - when both motor and phonic tics are present for more than a year

A tic disorder not specified - tics are present but do not meet the criteria for any specific tic disorder

Causes of TS  Tourette Syndrome (TS) is a complex, neurological condition and it is not yet known what causes it. The condition is hereditary and a person with TS has, roughly, a 50 percent chance of passing on the gene to their children.

This doesn’t mean, though, Tourettes Action tells us,  that the child will inherit an identical form of TS. Their condition may be milder or more severe than their parents' and they may display different types of tics. So far no single gene has been convincingly identified, and exactly how TS is inherited is not clear.

TS and the brain  The genetics of TS is complex and many researchers believe that an individual can inherit a vulnerability to a spectrum disorder that includes TS, obsessive-compulsive disorder, and perhaps also attention deficit hyperactivity disorder.

Although the cause has not been established, it appears to involve an imbalance in the function of the neurotransmitters (chemical messengers in the brain), dopamine and serotonin. It is also likely to involve abnormalities in other neurotransmitter systems of the brain.

Brain scanning has revealed that there are some areas of the brain that appear to be different in individuals with TS, for example, some structures in the basal ganglia part of the brain, and in the frontotemporal brain areas.

Research has shown that different environmental factors may, in some cases, contribute to the onset or affect the severity of TS. These include pregnancy and birth-related problems such as complications during pregnancy, smoking during pregnancy, severe nausea and/or vomiting during the first trimester and premature low birth weight children.

PANDAS is an abbreviation for Paediatric Autoimmune Neuropsychiatric Associated with Streptococcal infections. This term describes a subset of children who have OCD and/or tic disorders such as TS, and in whom symptoms worsen following strep infections such as ‘strep throat’ and scarlet fever.

Symptoms  With PANDAS, children usually have a dramatic, ‘overnight’ onset of symptoms preceded by a strep throat infection. Symptoms might include motor or vocal tics, obsessions, and/or compulsions. Associated symptoms include mood changes, sleep problems, joint pain, urinary frequency and separation anxiety.

A sudden onset of symptoms is often followed by a slow, gradual improvement in symptoms. If they have another strep throat infection, symptoms can return just as suddenly and dramatically as they did before.

Cause  The exact cause behind this phenomenon is not yet known, although it is thought that the mechanism is similar to that of rheumatic fever – an autoimmune disorder triggered by strep throat infections. In the case of PANDAS and TS, it is believed that the antibodies produced to fight the strep throat infection mistakenly recognize and ‘attack’ a part of the brain called the Basal Ganglia, which is believed to be responsible for movement and behavior. Thus the antibodies interact with the brain to cause tics and/or OCD.

Treating Tourette Syndrome (TS) There is no cure and research into medical treatment is difficult because the severity of symptoms is very hard to measure objectively. Also, TS fluctuates naturally with or without treatment, making it difficult to say for sure how effective drug and behavioral therapy is; and individuals have hugely varied responses to medicines.

Different ways to treat TS  There are, however, numerous reports of different drugs being used successfully in a small number of TS patients. Psychological therapies, most significantly Cognitive Behavioural Therapy, are also having degrees of success with some people with TS. There are also on-going trials of a procedure called Deep Brain Stimulation in people with extreme cases of TS, which has reduced instances of tics in some patients, although the results so far are mixed.

To find out more detailed information on the treatment of TS visit the Tourettes Action website 

Listen to this weeks radio report

All material on this website is provided for your information only and may not be construed as medical advice or instruction. No action or inaction should be taken based solely on the contents of this information; instead, readers should consult appropriate health professionals on any matter relating to their health and well-being.