What Is Tourette's Syndrome

Overview

Tourette’s syndrome (TS) is a neurological disorder characterized by involuntary, repetitive sounds, twitches or movements known as tics. For example, an unwilling grunting sound or repetitive blinking. Individuals with tics cannot help their bodies stop doing them. It is usually a childhood disorder. TS can significantly impact a person’s daily life such as academic performance and social functioning and the severity of the disorder can vary between individuals. In this article, you will learn about the causes, signs, symptoms, and management of the disorder.¹

Causes of tourette's syndrome

The exact causes of TS are not currently known. However, both genetic and environmental factors are believed to play a role. Research has demonstrated that TS is greatly impacted by genetic factors.² One of the genes that has been associated with a small percentage of individuals with TS is the SLITRK1 gene. This gene is responsible for activating a protein in the brain which in turn plays a role in nerve cells’ development. The SLITRk1 protein is involved in the growth of axons and dendrites allowing communication between adjacent cells. Nevertheless, the mechanisms by which this gene leads to TS development are unclear.³ Mutations in the CNTN6 and NRXN1 genes may also play a role in TS. Both these genes regulate the formation of nerve connections.⁴ Researchers suspect the involvement of other genes as well in TS.³ However, a study showed that the concordance rate for monozygotic and dizygotic twins was 53% and 8% respectively⁵ highlighting that although genes may be involved in TS development, environmental factors may also play a role.⁴

The effect of environmental factors in TS may begin as early as the perinatal period and continue throughout the lifespan of an individual. Environmental factors that can trigger the manifestation of TS or worsen its symptoms include infections, noxious exposure during pregnancy, birth complications, exposure to medication during pregnancy, older paternal age, birth weight, maternal smoking and alcohol consumption, and maternal stress during pregnancy.⁶ All these factors may play a role in the development of TS. However, more research is needed to investigate the exact involvement of genetic and environmental factors together. 

Signs and symptoms of tourette's syndrome

The hallmark symptom of TS is tics. Tics are brief and sudden movements or sounds and normally appear in early childhood around the ages of 2 to 14. Tics can be divided into two categories (motor and vocal) and their severity may range from mild to severe.^7,8

Motor, simple tics:

• Eye blinking
• Eye darting
• Eye rolling
• Nose twitching
• Mouth movement
• Grimacing 
• Head jerking
• Shoulder shrugging

Motor, complex tics:

• Hopping
• Smelling/touching objects/people
• Bending
• Twisting
• Obscene gesturing
• Stepping in a particular pattern
• Observed movements’ repetition

Vocal, simple tics:

• Grunting
• Coughing
• Barking
• Whistling 
• Tongue clicking 
• Animal sounds
• Clearing throat

Vocal, complex tics:

• Repeating others’ phrases
• Repeating their own phrases
• Using swear words

Tics may not be dangerous to an individual’s overall health but can get worse in periods of high anxiety and stress. Individuals with TS may also have other mental illnesses:^4,8

• OCD (obsessive-compulsive disorder)
• ADHD (attention deficit hyperactivity disorder)
• Depression
• Anxiety
• Learning disabilities
• Insomnia
• Sensory processing problems
• Social functioning deficits

Management and treatment for tourette's syndrome

There is no available cure for TS, however, there are several treatments that can help you manage your symptoms. Most people have mild symptoms and may not require treatment. If your symptoms interfere with your daily functioning and activities, medications may be prescribed to reduce these symptoms or symptoms caused by other disorders.^7,8,9

• Medications that block dopamine
• Botulinum injection in the muscle may help a tic
• Central adrenergic inhibitors can help in controlling behavioural symptoms
• ADHD medications can help in increasing attention
• Antiseizure medications 
• Antidepressants

Apart from medication, several types of therapy may also be used for individuals with TS to help them manage their symptoms:^7,8,9

• Behavioral therapy: can help reduce tics’ severity, impact, and number
• Habit reversal: learn to identify tics and learn to do another behaviour at the same time as the tic
• Cognitive Behavioral Intervention for Tics (CBIT): includes relaxation techniques and education about tics
• Deep brain stimulation: if no other techniques work, a device delivers electrical stimulation to targeted areas in order to control movement

Diagnosis of tourette's syndrome

The diagnosis for TS is usually based on your symptoms and signs. There is no specific test used to diagnose TS. However, to rule out other health issues blood tests and MRI are recommended. In order to get a full diagnosis for TS, the doctor will ask you questions about your tics and you should have all of the following criteria:^4,7,8,9

• Both vocal and motor tics (not necessary at the same time)
• Tics should occur at several times during a day for over a year
• Tics should appear before the age of 18
• Not taking any medication that might be causing the tics
• Tics should change over time (complexity, location, severity, type, frequency)

FAQs

Can tourette's syndrome be prevented

Since the cause is unknown, there is no known, successful way to prevent TS. Nevertheless, several measures can be taken to reduce its impact on the life of the individual such as managing stress, healthy lifestyle, medication, and therapy.^4,7,8,9

Who are at risks of tourette's syndrome

Risk factors of TS include:⁷

• Sex: males are more likely to have TS than women
• Family history

How common is tourette's syndrome

TS occurs in all populations worldwide. The prevalence of the disorder is estimated to be 1-10 every 1,000 children. However, it has been suggested that almost half of children with TS may not get a diagnosis. TS is more prevalent in males than females.^3,9

What should I expect if I have tourette's syndrome

If you have TS you should expect your doctor to ask you many questions regarding your symptoms. You should be ready to answer these questions quickly as the time with your doctor may be limited so it is best to write down all of your symptoms. Questions you may be asked include:⁷

• When did your symptoms appear?
• What worsens your symptoms?
• What improves your symptoms?
• Are the symptoms continuous?
• How severe are your symptoms?

When should I see a doctor

If your child presents involuntary sounds or movements you should contact your paediatrician. Note that not all tics mean that someone has TS, several children develop tics that can later go away. But, when a child displays unusual behaviour it is crucial to contact your doctor in order to rule out health problems.^7,8

Summary

Tourette’s syndrome is a neurological disorder that appears in childhood characterized by tics (involuntary and repetitive movements or sounds). The exact causes of the disorder are not known but are thought to be a combination of genetic and environmental factors. Common risk factors include the male sex and family history. There is no cure for the disorder but there are several treatments to manage its symptoms such as medication and different types of therapy. It is important to seek medical attention at an early age to get an accurate diagnosis and improve the person’s daily life.