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Tourette syndrome (TS) - A Guide

Tourette syndrome (also called Tourette's syndrome) is a neurological condition that affects the brain and nervous system. It is characterised by a combination of involuntary noises and movements called tics

Introduction
Symptoms of Tourette Syndrome
Causes of Tourette Syndrome
Diagnosing Tourette SyndromeD
Treating Tourette Syndrome
Further Issues
Resources

Introduction

History of Tourette Syndrome

In 1825 the first case of TS was reported in medical literature with a description of the Marquise de Dampierre, a noblewoman whose symptoms included involuntary tics of many parts of her body and various vocalizations including coprolalia and echolalia.

19th century French neurologist Jean-Marc Itard described his patient as having motor tics, echolalia and coprolalia. His unfortunate patient, the Marquise de Dampierre, was a French noblewoman who developed motor tics at age 7 years and shortly thereafter developed involuntary vocalizations consisting of screams and strange cries. Several years later she developed coprolalia. With this host of problems, the Marquise was forced to live in seclusion and continued her involuntary cursing until her death at age 85.

Some 50 years after Itard's report, in 1885, another French neurologist Georges Gilles de la Tourette produced a detailed account of several patients with a similar condition, including the Marquise in her later years, that clearly established this entity. Jean Martin Charcot, one of the leading European neurologists of the 19th century and Gilles de la Tourette's supervisor at the Salpetriere, attached his pupil's name to this syndrome.

Georges Gilles de la Tourette was born (Georges Albert Edouard Brutus Gilles de la Tourette) in 1857, and died in 1904.

The syndrome usually starts during childhood and continues into adulthood. In many cases it runs in families and it is often associated with obsessive compulsive disorder (OCD) or attention deficit hyperactivity disorder (ADHD).

Books about Tourette Syndrome



Tourette Syndrome (The Facts)


Kids in the Syndrome Mix of ADHD, LD, Autism Spectrum, Tourette's, Anxiety, and More!: The one stop guide for parents, teachers, and other professionals


Coping with Tourette Syndrome: A Workbook for Kids with Tic Disorders

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Symptoms of Tourette Syndrome

If your child has tics, it does not necessarily mean that they have Tourette syndrome.

Children often develop tics before growing out of them after several months. These are known as transient tics.

For tics to be classified as Tourette syndrome, they have to be present for at least a year and include at least one vocal tic.

Tics can be:

  • vocal (sounds) – such as grunting, coughing or shouting out words
  • physical (movements) – such as jerking of the head or jumping up and down

Tics can also be:

  • simple – for example, making a small movement or uttering a single sound
  • complex – for example, making a series of movements or speaking a long phrase

Most people who are diagnosed with Tourette syndrome have a combination of physical and vocal tics, which can be both simple and complex.

Simple vocal tics

Examples of simple vocal tics include:

  • grunting
  • clearing the throat
  • coughing
  • screaming
  • sniffing
  • squeaking
  • blowing



Simple physical tics

Examples of simple physical tics include:

  • blinking
  • jerking the head
  • twitching the nose
  • teeth grinding
  • rolling the eyes
  • twisting the neck
  • rotating the shoulders

Complex vocal tics

Examples of complex vocal tics include:

  • repeating other people’s phrases (echolalia)
  • repeating the same phrase over and over again (palilalia)
  • swearing loudly or shouting inappropriate words and phrases (coprolalia)

Swearing is often associated with Tourette syndrome, but it is actually a fairly uncommon symptom that affects a minority of people with the syndrome.

Complex physical tics

Examples of complex physical tics include:

  • shaking the head
  • hitting or kicking objects
  • jumping
  • shaking
  • touching themselves or others
  • copying the movements of others (echopraxia)
  • making obscene gestures, such as giving someone 'the finger' (copropraxia)

Premonitory sensations

Most people with Tourette syndrome experience uncomfortable or unusual feelings before having a tic. These feelings are known as premonitory sensations.

Premonitory sensations are only relieved after the tic has been carried out, in a similar way to how an itch can only be relieved by scratching it.

Examples of premonitory sensations include:

  • a burning feeling in the eyes that feels as if it can only be relieved by blinking
  • tension in a muscle that can only be relieved by twitching or stretching the muscle
  • a dry or sore throat that can only be relieved by grunting or by clearing the throat
  • an itchy joint or limb that can only be relieved by twisting the joint or limb

Pattern of tics

If your child has Tourette syndrome, their tics will probably tend to follow a set pattern. They may be worse during periods of:

  • stress
  • anxiety
  • tiredness
  • illness
  • nervous excitement
  • relaxation after a busy day

On the other hand, the tics are often reduced when they are doing an enjoyable activity that involves a high level of concentration such as reading an interesting book or playing competitive sports.

You may find that your child is able to control their tics when they are in situations where they would be particularly noticeable, such as in a school classroom. However, controlling tics can be difficult and tiring over prolonged periods of time.

Many children with Tourette syndrome often experience a sudden 'release' of tics after trying to suppress them – for example, after returning home from school.

When to seek medical advice

You should contact your GP if either you or your child starts experiencing tics.

Many children have tics for several months before growing out of them, so a tic does not necessarily mean your child has Tourette's syndrome.

However, symptoms such as tics do need to be investigated.

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Causes of Tourette Syndrome

The cause of Tourette syndrome is unknown. However, it is thought to be linked to problems with an area of the brain known as the basal ganglia.

Basal ganglia

The basal ganglia are a group of specialised brain cells located deep inside the brain. The cells help regulate the body's movements.

Research suggests that the basal ganglia may also play a role in higher brain functions such as motivation and decision making.

In people with Tourette syndrome, it appears that tics are the result of a temporary problem that occurs inside the basal ganglia and disrupts the decision-making process.

The person suddenly develops an unconscious urge to perform an action (the tic) that the conscious mind regards as both unwanted and unexplained.

It is not known what actually goes wrong with the basal ganglia. One theory suggests that excessive levels of a naturally occurring chemical called dopamine, which can have powerful effects on the brain, could be responsible.

Alternatively, the dopamine levels could be normal in people with Tourette syndrome, but they may be particularly sensitive to its effects.

Brain imaging studies have also shown that the structure of the basal ganglia is different in people with Tourette syndrome. However, it is unclear whether these changes are due to a dopamine imbalance or sensitivity, or the cause of it.

Possible triggers

As with the cause of Tourette syndrome, it is not known what triggers it. There are several theories, which are outlined below.

Genetics

Genetics appears to play a part in some cases of Tourette syndrome, as it often runs in families.

Further evidence suggests that if one identical twin develops Tourette syndrome, there is about a 1 in 2 chance that the other twin will also develop it.

It may be that a genetic mutation disrupts the normal development of the brain, triggering the symptoms of Tourette syndrome. A genetic mutation is where the instructions contained in all living cells become scrambled in some way.

Read more about genetics.

Childhood infection

Another theory is that Tourette syndrome may be linked to a childhood infection by streptococcal bacteria (bacteria that usually cause a sore throat).

In an attempt to fight off the infection, the immune system produces antibodies (proteins). The antibodies may interact with brain tissue, affecting the brain's functioning.

Following a throat infection, some children have developed the symptoms of Tourette syndrome, as well as the symptoms of obsessive compulsive disorder (OCD).

Some doctors believe that this may be a separate condition in its own right and have called it 'paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections', or PANDAS for short.

However, the concept of PANDAS is controversial because research findings have been inconsistent. It may be that it is only responsible for a small number of Tourette syndrome cases. Further research is needed to clarify the issue.

In the meantime, the same treatments are recommended as those used in ordinary tic disorders and Tourette syndrome.

Plasticity

The human brain has a high degree of what neurologists call 'plasticity'.

The various networks and pathways between brain cells can change and adapt over time into new networks and pathways.

One possibility is that many people grow out of Tourette syndrome because their brain effectively 'rewires' itself to compensate for problems with an area of the brain known as the basal ganglia.

Once the rewiring is complete, a person's symptoms either significantly improve or disappear altogether.

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Diagnosing Tourette Syndrome

The first stage in diagnosing Tourette syndrome is to rule out other possible causes of your child’s symptoms.

Other possible causes include:

  • allergies – if they are sniffing and coughing
  • vision problems – if they are blinking more than usual

It is also necessary to rule out other conditions that can cause tic-like behaviours, such as:

  • autistic spectrum disorder – a developmental disorder that causes problems with social interaction, learning and behaviour, and may cause mannerisms or stereotypies (repetitive movements) that can be mistaken for tics
  • dystonia – a condition that causes involuntary muscle spasms

To help rule out these conditions, your child may be referred to a number of experts, such as:

  • a neurologist – a doctor who specialises in treating conditions that affect the brain and nervous system
  • a psychiatrist – a doctor who specialises in treating mental health conditions
  • an educational or clinical psychologist – healthcare professionals who work with children who have learning, developmental or behavioural difficulties

Brain-imaging scans can also be used to check for any brain or nervous system abnormalities that could suggest a neurological cause for your child’s symptoms, other than Tourette syndrome. However, most children with tics or Tourette syndrome do not require a brain scan.

Scans that can be used include a:

  • computerised tomography (CT) scan – where a series of X-rays are taken to build up a detailed 3D image of the brain
  • magnetic resonance imaging (MRI) scan – where strong magnetic fields are used to produce a detailed image of the inside of the brain

Confirming the diagnosis

There is currently no single test for Tourette syndrome. A diagnosis can only be made by assessing your child’s symptoms to see whether they follow the pattern that is usually associated with the syndrome.

A confident diagnosis of Tourette syndrome can usually be made if your child:

  • has symptoms that are not being caused by other medical conditions or any medication you are taking
  • started having tics before 18 years of age
  • has had several physical tics and at least one vocal tic
  • has tics that occur many times during the day, virtually every day
  • has been having tics for at least a year

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Treating Tourette Syndrome

There is no cure for Tourette's syndrome, but treatment can help to control the symptoms.

There are several treatment options available for people with Tourette's syndrome.

Treatment plan

The first and most important part of treatment for Tourette's syndrome is to ensure that you, your child and, if needed, your child’s teachers, friends and other family members all have good information and knowledge about the condition. This includes:

  • being able to explain Tourette syndrome to others if asked about tics
  • understanding that tics are not ‘put on’
  • understanding that tics come and go and that sometimes they may be better than at other times
  • knowing that having tics does not generally stop you doing anything you want to do
  • knowing that treatments may help to relieve tics, but are unlikely to make them go away completely
  • being aware that most people ‘outgrow’ tics by the time they reach adulthood

Next, the treatment plan for tics could involve one or more of the following:

  • treatments that do not involve taking medication – such as behavioural therapy
  • medication – the three types of medication that may be prescribed are alpha2-adrenergic agonists, muscle relaxants and dopamine antagonists
  • surgery – in very rare, severe cases, surgical techniques may be suitable

Non-medical treatments alone, such as behavioural therapy, may be needed if the tics are relatively mild and infrequent.

In cases where a person's tics are more severe and disrupt day-to-day activities, a combination of therapy and medication may be recommended.

Surgery is usually only recommended as a 'treatment of last resort' if the tics are very severe and fail to respond to other treatment.

The doctor in charge of your or your child’s care, usually a neurologist (a brain and nervous system specialist), will recommend what they think is the best treatment option. However, the final decision will be yours.

If your child is old enough to understand fully the implications of their decision, they will be asked to decide what treatment they would prefer.

Detecting and treating mental health problems associated with Tourette syndrome, such as obsessive compulsive disorder (OCD), attention-deficit hyperactivity disorder (ADHD), depression and anxiety, is often seen as more important than treating the tics.

Behavioural therapy

Behavioural therapy is a widely used non-medical treatment for Tourette syndrome. It is a type of psychological treatment that is designed to change the pattern of your or your child’s behaviour.

Habit reversal

Habit reversal is a type of behavioural therapy that has proved successful in treating Tourette syndrome. It is based on the following two principles:

  • people with Tourette syndrome are often unaware of their tics
  • tics are used to relieve uncomfortable feelings, called premonitory sensations, that build up before tics

The first stage is to monitor the pattern and frequency of the tics, and identify any sensations that are triggering them. The next stage is to find an alternative, less noticeable method of relieving the premonitory sensations instead of a tic. This is known as a competing response.

For example, your child may experience an unpleasant sensation in their throat that causes them to grunt. Therefore, the next time your child feels the unpleasant sensation, they are asked to take a series of deep breaths rather than grunting, to try to relieve the sensation.

Habit reversal is often combined with relaxation therapy. Relaxation techniques, such as deep breathing or visualisation (thinking about something pleasant as a distraction), can help prevent feelings of stress and anxiety, which can often make tics worse.

Exposure and response prevention (ERP)

Exposure and response prevention (ERP) involves increasing exposure to the urge to tic in order to suppress the tic response for longer. This works on the theory that you get used to the feeling of needing to tic until the urge, and any related anxiety, decreases in strength.

Medication

Alpha2-adrenergic agonists

Alpha2-adrenergic agonists are usually recommended for treatment of mild to moderate symptoms of Tourette syndrome.

This type of medication is thought to stabilise levels of a brain chemical called norepinephrine. This is thought to decrease the risk of the basal ganglia misfiring and triggering tics.

Clonidine is the alpha2-adrenergic agonist that is widely used to treat Tourette syndrome.

Common side effects of clonidine include:

  • drowsiness
  • headaches
  • dizziness
  • fatigue
  • constipation
  • diarrhoea
  • a dry mouth
  • difficulties sleeping

These side effects are usually mild and should improve when the body gets used to the medication.

Muscle relaxants

Muscle relaxants have been shown to be effective in helping control tics, particularly physical tics.

Baclofen and clonazepam are two muscle relaxants that are sometimes used to treat Tourette syndrome. You or your child may feel drowsy and dizzy after taking these.

Adults being treated with muscle relaxants should not drive or use tools or machinery if they feel dizzy or drowsy. You should also avoid drinking alcohol while taking muscle relaxants because it could make you feel very ill.

Dopamine antagonists

Dopamine antagonists are the most effective type of medication for preventing tics. However, they can cause a wide range of side effects, so will only be recommended in cases where the symptoms are particularly severe or fail to respond to other medications.

Dopamine antagonists are given orally (as a tablet) or by injection. They work by blocking the effects of dopamine on the brain. Dopamine is a chemical in the brain thought to be associated with tics.

There are two main types of dopamine antagonists. They are:

  • first generation dopamine antagonists –that were developed in the 1950s
  • a newer generation of dopamine antagonists – that were developed in the 1990s

Both the older and newer dopamine antagonists have side effects, although not everyone experiences them, and their severity differs from person to person.

The side effects of the older dopamine antagonists include:

  • drowsiness
  • shaking
  • trembling
  • muscle twitches
  • spasms

Side effects of both the older and newer dopamine antagonists include:

  • weight gain
  • blurred vision
  • constipation
  • a dry mouth

The newer generation of dopamine antagonists are usually recommended because they are less likely to cause side effects. However, they are not suitable or effective for everyone.

Adults with Tourette syndrome who are being treated with dopamine antagonists may also experience a loss of libido (decreased sex drive).

If your child has been prescribed a dopamine antagonist, and they are finding the side effects particularly troublesome, you should contact the doctor in charge of their care. There may be an alternative that your child will be able to tolerate better.

Aripiprazole is the newest type of dopamine medicine that seems to be helpful and appears to have fewer side effects. Although there has not yet been a large clinical trial of this medicine for Tourette syndrome, it has been used successfully in many people with tics.

An experienced doctor may be able to offer aripiprazole as a treatment option.

Surgery and deep brain stimulation (DBS)

Surgery is usually regarded as a 'treatment of last resort' for people with severe Tourette syndrome that has failed to respond to other treatments. It is usually only recommended for adults.

The aim of surgery is to make a small ‘break’ in some of the pathways in the brain that may be responsible for tics. The region of the brain that is usually operated on is called the limbic system, although several different areas have been targeted.

Surgery has largely been replaced with deep brain stimulation (DBS). This is a relatively new technique that, like surgery, has been used to treat very severe cases of Tourette syndrome where other treatments have failed.

DBS involves permanently implanting electrodes (small metallic discs) in the parts of the brain that are known to be associated with Tourette syndrome.

The electrodes are attached to small generators that are implanted elsewhere in the body. The generators send electronic pulses to the electrodes, which stimulate different parts of the brain. By stimulating certain areas of the brain the symptoms of Tourette syndrome can often be controlled.

Initial results of DBS have been encouraging, with some people showing a substantial reduction in their tics that lasted for more than five years. DBS appears to be most effective when combined with behavioural therapy (see above).

However, both limbic system surgery and DBS have only been used on a small number of people with Tourette syndrome, and there is not yet sufficient evidence to justify their use as standard treatments for the syndrome.

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Further Issues

Tourette's syndrome is often associated with psychological and behavioural problems, as well as learning difficulties.

However, Tourette's syndrome does not usually affect a person's intelligence.

Obsessive compulsive disorder

Up to 60% of children with Tourette’s syndrome also develop obsessive compulsive disorder (OCD).

OCD is a long-term mental health condition where a person has obsessive thoughts and compulsive behaviours.

In children with Tourette’s syndrome, the symptoms of OCD usually take the following forms:

  • constantly checking things – for example, checking windows are locked or the door is not left open
  • a desire for order and symmetry – for example, their toys have to be lined up on the shelf in a specific way (if the order is disrupted, the child may become very upset)
  • hoarding – holding on to objects that have no real value, such as bus tickets or flyers for takeaways
  • cleaning – constantly cleaning due to an obsessive fear of disease and contamination (this often takes the form of compulsive hand-cleaning)

A physical tic and compulsive behaviour may be combined. For example, the child might constantly pick up an object and then place it down, or repeatedly open and close a door.

OCD is treated using a combination of medication, such as selective serotonin reuptake inhibitors (SSRIs) and psychological therapy, such as behavioural therapy.

With treatment, most people's symptoms will improve and some people will achieve a complete cure.

Read more about treating OCD.

Attention deficit hyperactivity disorder (ADHD)

Attention deficit hyperactivity disorder (ADHD) is another condition that often affects children with Tourette’s syndrome. It is thought to affect up to 70% of children with the syndrome.

ADHD is a behavioural condition causing problems with attention span, ability to control impulses, and ability to concentrate and plan ahead.

Children with Tourette’s syndrome and ADHD usually find it very difficult to focus on specific tasks for a prolonged period of time and are often easily distracted.

Behavioural problems

Other behavioural problems that can affect children with Tourette's syndrome include:

  • irritability
  • anxiety
  • antisocial behaviour
  • flying into sudden rages
  • self-harming behaviour (less commonly)
  • inappropriate behaviour to others

As a child gets older, this inappropriate behaviour can often take the form of making inappropriate sexual remarks or acting in a sexually aggressive manner.

These types of problems often improve once a child begins treatment for Tourette’s syndrome and their tics start to be better controlled.

Learning difficulties

Tourette’s syndrome can be associated with learning difficulties, particularly if a person also has ADHD or OCD.

Many people with Tourette’s syndrome find it difficult to learn through habit (this is how a child usually learns to read, for example). This is because the basal ganglia is the part of the brain that controls habit learning, and it is also the part most associated with Tourette’s syndrome.

Therefore, children with Tourette’s syndrome may have difficulty mastering skills and activities that other children pick up as a matter of routine, such as reading, writing and simple maths (adding and subtracting).

Some children with Tourette’s syndrome may require additional specialised educational support. Your local education authority (LEA) can arrange an assessment of your child’s educational requirements before drawing up a plan to meet their needs.

Resources

Tourettes Action - Find out about available treatments and support.

Tourette Scotland - Support, advice, information

Tourette sydrome at Wikipedia

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