Tourette Syndrome

Tourette Syndrome

What is Tourette syndrome?

Tourette’s syndrome, pronounced ‘too-RET’, which is also commonly known as Tourette’s, is a neurological disorder. This means that it is a condition that affects the nervous system. It is referred to as a ‘tic’ syndrome due to the condition being characterised by the patient having involuntary motor or vocal tics. Tics are spasmodic contractions of the person’s muscles. A vocal tic is characterised by a vocal outburst and a motor tic involves an action of some kind.

A person who has Tourette’s will experience these tics due to abrupt and intermittent twitches of a specific group of muscles. Tics may include grunting, sniffing, blinking, throat clearing, shoulder and head movements to name a few. Vocal outbursts can include repeating certain words, some of which may seem offensive, or making a strange noise at inappropriate moments.

The exact cause of the condition is unknown however, it does not affect the patient’s life expectancy or intelligence in any way (although it may cause learning difficulties for some).

Tourette’s is known to affect four times as many males than females.

The symptom of tics tends to appear between the ages of two and 15, while the average age of Tourette’s becoming apparent is six years old. The symptoms have a tendency to be the most severe during the adolescent years.

Tics can be simple, meaning that they only affect one or two parts of the body, such as blinking, grunting or making a strange face. Or they may be complex often involving multiple word repetition, moving many parts of the body such as jumping or vocalising swear (curse) words.

The patient may experience a sensation of tingling or tension before a motor tic occurs, and by completing the motor tic, this sensation is relieved and goes away. He/she may be able to postpone the occurrence of the tic for a short period, but they are unlikely to stop them from occurring completely.

Issues with paying attention, sitting still as well as completing tasks are symptoms often associated with ADHD (attention deficit hyperactivity disorder) and are often displayed by those with Tourette’s. Doctors are still uncertain as to whether there is a link between the two disorders and research is still being conducted.

Unfortunately, there is no cure for Tourette syndrome, however, there are various treatments available that are able to ease and reduce the effects of the associated tics. Some people may need treatment more than others, this depends on how bothersome their tics are.

It is important to note that these ‘tics’ are out of the patient’s control. Some tics may be more severe than others and occur more frequently. Less severe tics may not be as troublesome for the patient and tend to become more controlled and lessen as the patient becomes an adult. Tics may even disappear completely as the patient reaches adulthood.

This article explores what you need to know regarding Tourette syndrome. Taking you through the causes, symptoms, risk factors, diagnosis and treatment. It is vital to note that this article should only serve as a guideline and not as a professional medical opinion.

What are the symptoms of Tourette Syndrome?

As discussed, the defining symptoms of Tourette’s are tics. These are brief, sudden and intermittent sounds or movements, ranging from mild to severe. Severe tics have been known to significantly impact the sufferer’s communication, social life, quality of life and ability to perform daily tasks.

Tics can be divided into two categories:

  • Brief, sudden and repetitive tics which are known as simple tics – these are limited to only a few muscle groups.
  • Distinct and coordinated patterns which are known as complex tics – these involve a number of muscle groups.

Tics can be vocal (sound) or motor (movement). Motor tics tend to appear before vocal tics. Each patient may experience tics differently, ranging from vocal and motor tics which may also differ in their level of severity.

Before the onset of a tic, the patient is likely to experience a tingling or uncomfortable sensation in their body, this is known as a premonitory urge. This often feels like a tingle, itch or a feeling of tension. By expressing this through a tic, it brings relief to the sensation. Patients who are able to hold back or completely stop a tic, experience a great deal of difficulty in doing so.

The common simple and complex vocal (sound) tics of Tourette syndrome are as follows:

Simple vocal tics:

  • Coughing
  • Grunting
  • Barking
  • Throat clearing

Complex vocal tics:

  • Repeating other people’s words or phrases
  • Repeating one’s own words or phrases
  • Using swear (curse), vulgar or offensive words

The common simple and complex motor (movement) tics of Tourette syndrome are as follows:

Simple motor tics:

  • Blinking eyes
  • Jerking head
  • Shrugging shoulders
  • Twitching nose
  • Darting eyes
  • Moving mouth

Complex motor tics:

  • Smelling or touching objects
  • Hopping
  • Twisting or bending
  • Obscene gesturing
  • Repeating movements that have been observed
  • A certain pattern of stepping

Tics can also have the following factors to them:

  • They may change and develop over time
  • They may happen when the patient is sleeping
  • They may worsen during the early adolescent years and then improve once the patient matures into adulthood
  • They may worsen when the patient is stressed, tired, agitated, anxious or ill
  • They may vary in severity, type and frequency

When to see a doctor

As stated, when a child with Tourette’s is roughly six years old, tics usually become apparent. Children are therefore first diagnosed with the condition around this age. The condition is then monitored and possibly treated as the child grows up. If a child experiences tics associated with display movements and sounds that are involuntary, it is best to consult with a paediatrician.

It is vital to note that not all tics are classified as Tourette syndrome. A lot of children tend to develop tics in their younger years, these seem to disappear within a few weeks or months. However, if the child is showing behaviour that is unusual on an ongoing basis, then it is important that the cause of the behaviour is identified by a medical professional in order to rule out any other possible conditions that may be more serious.

What are the causes, risk factors and complications of Tourette syndrome?

Causes of Tourette’s

  • Brain abnormalities
  • Genetics

Tourette’s is linked to an area in the brain known as the basal ganglia. This part of the brain aids in controlling the movements of the body. Tourette’s has also been linked to a number of other areas in the brain, however, the basal ganglia has been found to be the most prominent.

If changes occur in the basal ganglia, these may impact the chemicals and nerve cells that are responsible for carrying messages in the brain. These nerve cells are called neurotransmitters. These include serotonin, dopamine and norepinephrine. Research has shown that issues in this network of the brain may have a role to play in Tourette’s.

Researchers and doctors are still unsure as to what causes these issues in the brain in the first place, but genes are thought to play a role in bringing about these changes.

If a person has someone in their family with Tourette’s, they stand a higher chance of developing the condition themselves which points to the condition being hereditary. However, research has shown that patients in the same family, often experience different symptoms, which makes the genetic component a complex one.

However, researchers believe that it is likely that Tourette’s may have more than just one cause and environmental risk factors such as maternal smoking or alcohol consumption during pregnancy, birth complications, low birth weight and group A strep infection during childhood are being researched as possible causes of Tourette’s

It is important to note that Tourette’s is an extremely complex condition and it cannot be prevented. The condition’s cause involves abnormalities in various parts of the brain and the electrical circuit responsible for connecting them. As previously mentioned, an abnormality may exist in the basal ganglia, thus affecting the patient’s movements.

 Neurons in the brain

Risk factors of Tourette’s

There are two main risk factors for Tourette’s, these being:

  • Sex – Four times more males are diagnosed with Tourette syndrome than females.
  • Family history (genes) – Tic disorders and Tourette’s have been found to run in families. Having someone in the family with the condition may increase a person’s risk of developing the condition. However, the symptoms differ between each person.

Complications of Tourette’s

Someone with Tourette’s often lives a healthy and active life. However, there are certain issues with the condition that involve social and behavioural challenges that have been known to harm the self-image and social life of an affected individual.

Further complications come into play regarding common co-occurring conditions with Tourette’s. These conditions consist of neuropsychiatric and neurodevelopmental disorders. Some of these may exist before the condition develops and create further impairment regarding the tics of Tourette’s. In other cases, the condition may only develop after the patient has been diagnosed with Tourette syndrome.

Co-occurring conditions can include:

  • ADHD (attention deficit hyperactivity disorder) – Symptoms of this condition typically include the patient feeling restless, being hyperactive, impulsive and experiencing difficulty in concentrating on one thing at a time.
  • OCD/OCB (obsessive-compulsive disorder/behaviours)Unwanted, repetitive and intrusive thoughts and behaviours are characteristics of OCD. There are several types of obsessive urges or thoughts, these can include the patient having to do things ‘just right’ or having aggressive or even sexual thoughts that may appear out of the patient’s control. These thoughts have been known to lead to compulsions in having to do something they may not want to do. Or performing something in a certain way. The severity of these compulsions often varies from one patient to another. An example may be that they feel the excessive need to fold their clothes or arrange their cupboard in a certain way, a more severe compulsion may involve the patient feeling as though they have to commit a crime.
  • Learning difficulties – Having issues with writing (dysgraphia), reading, mathematics and/or processing information that has no relation to the patient’s general intelligence.
  • Behavioural issues – Rage, aggression, committing socially inappropriate acts (disinhibition) or expressing oppositional defiance.
  • Anxiety – Excessively worrying, feeling fearful and experiencing separation anxiety.
  • Mood issues – Experiencing periods of mood elevation or depression that result in the person’s behaviour or functioning being impaired. Mood issues are normally vastly different to the person’s normal behaviour.
  • Issues with social functioning and social skills – Having trouble with developing social skills and maintaining relationships with friends and family, or acting in an inappropriate manner.
  • Sleeping issues – Having trouble staying or falling asleep, sleep walking or talking and bedwetting.

How is Tourette Syndrome diagnosed?

Tourette syndrome does not have a specific test for diagnosis. The diagnosis of the condition is based on the patient’s history of symptoms and signs.

The diagnosis of the condition can sometimes be overlooked due to the signs mimicking those of other conditions. If a patient is always blinking their eyes, they may be experiencing issues with their vision, the same thinking goes for constant sniffing being linked to allergies.

 Diagnosis Tourette's

The following criteria are used to diagnose Tourette's:

  • Vocal tics and motor tics are both present, however, they do not have to appear at the same time.
  • The tics started before the patient turned 18 years old.
  • The tics are not the result of other medications, another condition or substances ingested.
  • The tics occur a number of times a day, basically every day for more than an entire year.

If a child displays symptoms of Tourette’s, the parent may be advised by their doctor to see a healthcare professional who specialises in diseases of the nervous system – known as a neurologist.

In order to rule out possible other causes of the tics, the neurologist may conduct the following tests:

  • Neuroimaging tests:
    • MRI (magnetic resonance imaging) – This test utilises radio waves and powerful magnets to create pictures of structures inside the body.
    • CT (computerised tomography) – This test is a powerful X-ray that creates extremely detailed images of the patient’s organs.
    • EEG (electroencephalogram) – This is a test that detects the electrical activity in the patient’s brain by using electrodes (flat metal discs) that are attached to the patient’s head.
  • Blood tests

The doctor will ask the parent (if dealing with a minor) or patient about the symptoms experienced. An accurate diagnosis will require both one vocal tic and one motor tic to have occurred for at least a year.

As mentioned, other conditions will need to be ruled out, the most common of these include:

  • ADHD (attention deficit hyperactivity disorder)
  • OCD (obsessive-compulsive disorder)
  • Learning disabilities
  • Sleeping disorders
  • Anxiety

Questions the doctor may ask

  • What signs were noticed that led to booking an appointment with the doctor?
  • Does the patient often move their body in a way that is involuntary? How long have these involuntary movements been happening?
  • Does the patient ever say things without the intention to? When did these involuntary vocal tics start?
  • Does the patient have issues with focussing or often feel anxious?
  • Are there any members of the family who have the same symptoms?

Questions for the doctor

  • Will the symptoms go away ad how long will they last?
  • Are there any more tests needed?
  • What is the treatment plan from here?
  • Are there any side effects to be aware of regarding treatment?
  • Are there any specialists that need to be seen from here?
  • What can be done to help control or lessen the tics?

How is Tourette syndrome treated?

There is no cure for Tourette’s and the aim of the treatment is to help control the tics interfering with everyday functioning and activities. If the tics are not as severe, then treatment may not be needed.

Medication

The following medications can help to control the tics or aid in reducing the symptoms of co-occurring conditions:

  • Medications to lessen or block dopamine - Pimozide (Orap), haloperidol (Haldol) and Fluphenazine are known to aid in controlling tics by blocking or lessening the effects of dopamine in the brain. The side effects of these medications can include involuntary movements that are repetitive and/or weight gain. Dopamine is a neurotransmitter in the brain that aids in regulating emotional and movement responses.
  • Botox (botulinum) injections – Injecting Botox into the affected muscle may be able to help stop motor and vocal tics as Botox prevents muscle movement of the injected area.
  • ADHD (attention deficit hyperactivity disorder) medications – Stimulants including methylphenidate (Ritalin and Concerta being the most popular) and other medications that contain dextroamphetamine (Dexedrine and Adderall XR) have been found to help improve the patient’s concentration and attention span. However, in some patients with Tourette syndrome, certain ADHD medications have been found to make tics worse.
  • Anti-seizure medications – Medications such as topiramate (Topamax) have recently been shown to reduce the symptoms of Tourette’s. Side effects may include drowsiness and weight loss.
  • Central adrenergic inhibitors - These medications include guanfacine (Tenex) and clonidine (Catapres) – which are normally prescribed for hypertension (high blood pressure), and are able to help in controlling the behavioural symptoms such as rage and impulse control associated with Tourette’s. Side effects typically include drowsiness.
  • Antidepressants – These aid in controlling symptoms of anxiety and depression. Medications typically include Fluoxetine (Prozac and Sarafem).

Therapy

  • Behavioural therapy – Certain training techniques and strategies used by a medical professional can help ease the occurrence of tics and help the patient to control them. This includes Cognitive behavioural interventions for tics such as habit-reversal training. This helps the patient to identify when a tic is about to occur and how to move in a way that helps ease the tic, but not in a way that is obvious or inappropriate.
  • Psychotherapy – This kind of treatment has been seen to be extremely effective in not only helping patients deal with Tourette’s, but it also helps in co-occurring conditions such as anxiety, depression and ADHD. This involves having several sessions with a trained professional such as a psychiatrist or psychologist, vocalising how the condition is affecting the patient’s life and identifying how to deal with it.
  • DBS (deep brain stimulation) – When the patient is experiencing tics that are more severe and appear to be unresponsive to other means of treatment, a doctor may recommend DBS. This involves using a battery-operated medical device that is implanted in the patient’s brain. This delivers electrical stimulation to specific areas of the brain that control movement.

Why is support vital for Tourette syndrome?

Living with a condition like Tourette’s can often lead to the patient feeling isolated and alone. Suffering from involuntary tics that are not able to be controlled may result in the patient not wanting to take part in social settings.

However, there is support available for people in this situation. It is best to take advantage of the various treatments and resources available. Start by speaking to a doctor about local groups for support, there may also be options for group therapy available.

These support groups are able to help in dealing with social isolation and depression. Meeting people in a similar situation who face similar challenges and creating a bond with them can help ease feelings of isolation. Hearing other people’s personal stories and learning coping techniques from them can have a positive impact on the patient’s life.

If a child has Tourette’s, the parent should notify their school and teachers. Many children with the condition are often bullied at school. It is important for educators to be informed of the condition as they can help play a role in making the patient’s school life easier to deal with.

Motor and vocal tics may result in a child taking longer to complete tests or school work, teachers should give these children more time for completing tasks.

What to expect/outlook

In the majority of cases, children grow out of tics when they reach their late teens or early twenties. Some people may have the condition for their entire lives, but in these cases, symptoms lessen in severity and the patient is able to control them more.

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