Tourette syndrome

Tourette syndrome also known as Tourette disorder, Tourette’s disease or Chronic Motor and Vocal Tic Disorder, is a complex inherited disorder of the nervous system characterized by repetitive, sudden, and involuntary movements or noises called tics ¹⁾. Tics are repetitive movements or vocalizations such as grunting, coughing, blinking, sniffing, touching the ground, or tensing the abdomen. Tics — sudden, brief, intermittent movements or sounds — are the hallmark sign of Tourette syndrome. Symptoms of Tourette syndrome usually begin between the ages of 5 and 10 with mild, simple tics involving the face, head, or arms. Over time, tics become more frequent and increase in variety, involving more body parts such as the trunk or legs, and often become disruptive to activities of daily living. Severe tics might significantly interfere with communication, daily functioning and quality of life.

Tourette syndrome is named for Dr. Georges Gilles de la Tourette, the pioneering French neurologist who in 1885 first described the condition in an 86-year-old French noblewoman.

Tourette syndrome involves both motor tics, which are uncontrolled body movements, and vocal or phonic tics, which are outbursts of sound. Some motor tics are simple and involve only one muscle group. Simple motor tics, such as rapid eye blinking, shoulder shrugging, or nose twitching, are usually the first signs of Tourette syndrome. Motor tics also can be complex (involving multiple muscle groups), such as jumping, kicking, hopping, or spinning.

Vocal tics, which generally appear later than motor tics, also can be simple or complex. Simple vocal tics include grunting, sniffing, and throat-clearing. More complex vocalizations include repeating the words of others (echolalia) or repeating one’s own words (palilalia). Despite the fact that the involuntary use of inappropriate or obscene language (coprolalia) is possible, but these symptoms are rare among people with Tourette syndrome and are not required for a diagnosis of Tourette syndrome. The media often portray people with Tourette syndrome as involuntarily shouting out swear words called coprolalia or constantly repeating the words of other people called echolalia.

The severity and nature of the symptoms may wax and wane, and generally reach maximum intensity during adolescence. In most children with Tourette syndrome, the frequency and severity of symptoms have improved significantly by the time they reach adulthood. Whether this symptomatic improvement represents a resolution of the underlying disease pathology or improved compensatory mechanisms is not known.

In addition to frequent tics, people with Tourette syndrome are at risk for associated problems including attention deficit hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), anxiety, depression, and problems with sleep.

In addition, tics can:

• Vary in type, frequency and severity
• Worsen if you’re ill, stressed, anxious, tired or excited
• Occur during sleep
• Change over time
• Worsen in the early teenage years and improve during the transition into adulthood

Before the onset of motor or vocal tics, you’ll likely experience an uncomfortable bodily sensation (premonitory urge) such as an itch, a tingle or tension. Expression of the tic brings relief. With great effort, some people with Tourette syndrome can temporarily stop or hold back a tic.

Though a gene for Tourette’s syndrome has not yet been identified, there is strong evidence that it is an inherited disorder transmitted through one or more genes. Tourette syndrome can affect people of all ethnic groups.

Tourette syndrome doesn’t affect your intelligence.

Tourette’s syndrome is a common condition, and thought to affect 3-10 people out of every 1000. This may be a conservative estimate, since many people with very mild tics may be unaware of them and never seek medical attention. Age of onset of Tourette syndrome is usually from 2-15 years. Tourette’s syndrome is three to four times as likely to occur in boys as in girls. It is estimated that 200,000 Americans have the most severe form of Tourette syndrome, and as many as one in 100 exhibit milder and less complex symptoms such as chronic motor or vocal tics.

Children with Tourette syndrome are also at risk for having poor social relationships ²⁾. In addition to having tics, individuals with Tourette syndrome are at an increased risk for a number of co-occurring mental, emotional, or behavioral disorders, including attention-deficit hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), anxiety, depression, and autism spectrum disorder (ASD) ³⁾, ⁴⁾, which could affect the development and maintenance of social relationships and contribute to an overall lower social competence level compared to children without Tourette syndrome ⁵⁾, ⁶⁾, ⁷⁾.

There is no evidence to support the suggestion that events such as head trauma, encephalopathy or neuroleptic drugs predispose to Tourette syndrome.

Although Tourette syndrome can be a chronic condition with symptoms lasting a lifetime, most people with the condition experience their worst tic symptoms in their early teens, with improvement occurring in the late teens and continuing into adulthood.

There is no cure for Tourette syndrome, but it usually improves as the person gets older and does not shorten life span. Some people finds the tics go away as they enter adulthood.

Controlling tics

Some people can control their tics for a short while in certain social situations, like in a classroom. It requires concentration, but gets easier with practise.

Controlling tics can be tiring. A person may have a sudden release of tics after a day trying to control them, like after returning home from school.

Tics may be less noticeable during activities involving a high level of concentration, such as reading an interesting book or playing sports.

Living with Tourette syndrome

Tourette syndrome can cause problems with daily life, including learning, behaviour and sleep problems, anxiety and mood changes.

Treatment for Tourette syndrome uses medication to reduce particular symptoms, though most people with Tourette syndrome don’t need treatment or medication. Relaxation techniques can help reduce stress.

Children with Tourette syndrome

School may pose special challenges for children with Tourette syndrome.

To help your child:

• Be your child’s advocate. Help educate teachers, school bus drivers and others with whom your child interacts regularly. An educational setting that meets your child’s needs — such as tutoring, untimed testing to reduce stress, and smaller classes — can help.
• Nurture your child’s self-esteem. Support your child’s personal interests and friendships — both can help build self-esteem.
• Find a support group. To help you cope, seek out a local Tourette syndrome support group. If there aren’t any, consider starting one.

What is the best educational setting for children with Tourette syndrome?

Although students with Tourette syndrome often function well in the regular classroom, ADHD, learning disabilities, obsessive-compulsive symptoms, and frequent tics can greatly interfere with academic performance or social adjustment. After a comprehensive assessment, students should be placed in an educational setting that meets their individual needs. Students may require tutoring, smaller or special classes, and in some cases special schools.

All students with Tourette syndrome need a tolerant and compassionate setting that both encourages them to work to their full potential and is flexible enough to accommodate their special needs. This setting may include a private study area, exams outside the regular classroom, or even oral exams when the child’s symptoms interfere with his or her ability to write. Untimed testing reduces stress for students with Tourette syndrome.

Tourette syndrome facts

• Tourette Syndrome is a neurodevelopmental disorder that becomes evident in early childhood or adolescence. It is part of the spectrum of Tic Disorders and is characterized by motor and vocal tics.
• Tourette Syndrome and other Tic Disorders are not rare. The current estimates are that 1 out of every 160 children between the ages of 5-17 in the United States has Tourette syndrome and that 1 out of every 100 children has Tourette syndrome or another Tic Disorder.
• Some Tic Disorders may be transient, while others will persist into adolescence and adulthood. Tics come and go over time, varying in type, frequency, location, and severity. The first symptoms usually occur in the head and neck area and may progress to include muscles of the trunk and extremities. Motor tics generally precede the development of vocal tics and simple tics often precede complex tics. Most patients experience peak tic severity before the mid-teen years with improvement for the majority of patients in the late teen years and early adulthood. Approximately 10-15 percent of those affected have a progressive or disabling course that lasts into adulthood.
• It is common for people with Tourette Syndrome to be affected by another co-occurring condition.
  • Some co-occurring conditions are Attention Deficit Hyperactivity Disorder (ADHD) and Obsessive Compulsive Disorder (OCD). Problems with reading, writing, and arithmetic; and obsessive-compulsive symptoms such as intrusive thoughts/worries and repetitive behaviors. For example, worries about dirt and germs may be associated with repetitive hand-washing, and concerns about bad things happening may be associated with ritualistic behaviors such as counting, repeating, or ordering and arranging. People with Tourette syndrome have also reported problems with depression or anxiety disorders, as well as other difficulties with living, that may or may not be directly related to Tourette syndrome. In addition, although most individuals with Tourette syndrome experience a significant decline in motor and vocal tics in late adolescence and early adulthood, the associated neurobehavioral conditions may persist. Given the range of potential complications, people with Tourette syndrome are best served by receiving medical care that provides a comprehensive treatment plan.
• Tourette syndrome can be either severe or mild. Many people with very mild tics may not be aware of them and never seek medical help. Far fewer people have more severe forms of Tourette syndrome.
• The exact cause of Tourette syndrome isn’t known. Current research points to abnormalities in certain brain regions (including the basal ganglia, frontal lobes, and cortex), the circuits that interconnect these regions, and the neurotransmitters (dopamine, serotonin, and norepinephrine) responsible for communication among nerve cells. Given the often complex presentation of Tourette syndrome, the cause of Tourette syndrome is likely to be equally complex. It’s a complex disorder likely caused by a combination of inherited (genetic) and environmental factors.
• Genetic studies have indicated that Tourette syndrome is inherited as a autosomal dominant gene, with about a 50% chance of parents passing the gene on to their children, although the children may have no or fewer symptoms. Autosomal means that both males and females are affected, and dominant means that one copy of the gene is necessary to have the condition. This means that a parent with Tourette syndrome or a parent who has the gene for Tourette syndrome has a 50/50 chance, with each pregnancy, to pass the gene on to their child. However, multiple large studies have yet to identify a causal gene ⁸⁾.
• Mutations involving the SLITRK1 gene have been identified in a small number of people with Tourette syndrome ⁹⁾. The SLITRK1 gene provides instructions for making a protein that is active in the brain. The SLITRK1 protein probably plays a role in the development of nerve cells, including the growth of specialized extensions (axons and dendrites) that allow each nerve cell to communicate with nearby cells. It is unclear how mutations in the SLITRK1 gene can lead to Tourette syndrome ¹⁰⁾. Most people with Tourette syndrome do not have a mutation in the SLITRK1 gene. Because mutations have been reported in so few people with this condition, the association of the SLITRK1 gene with this disorder has not been confirmed. Researchers suspect that changes in other genes, which have not been identified, are also associated with Tourette syndrome.
• Tourette syndrome is associated with a non-genetic cause in 10 to 15 percent of children. Complications of pregnancy, low birthweight, head trauma, carbon monoxide poisoning, and encephalitis are thought to be associated with the onset of non-genetic Tourette syndrome.
• Boys with the gene(s) are three to four times more likely than girls to display symptoms of Tourette syndrome.
• Tourette syndrome can be triggered by abnormal metabolism (breakdown) of a chemical in the brain called dopamine.
• There is no cure for Tourette Syndrome, but thanks to years of dedicated research, there are various treatment options.

Tourette syndrome causes

The exact cause of Tourette syndrome isn’t known. Current research points to abnormalities in certain brain regions (including the basal ganglia, frontal lobes, and cortex), the circuits that interconnect these regions, and the neurotransmitters (dopamine, serotonin, and norepinephrine) responsible for communication among nerve cells. Given the often complex presentation of Tourette syndrome, the cause of Tourette syndrome is likely to be equally complex. It’s a complex disorder likely caused by a combination of inherited (genetic) and environmental factors.

Current research shows that Tourette syndrome is an inherited genetic condition, with children with Tourette syndrome having a family history 52% of the time ¹¹⁾, ¹²⁾. That means Tourette’s syndrome is passed on from parent to child through genes ¹³⁾, ¹⁴⁾, ¹⁵⁾. People with Tourette syndrome have a first-degree relative with Tourette syndrome at a rate 10 times the general population, and the ratio of concordance in monozygotic twins is 5:1 compared to dizygotic twins ¹⁶⁾. However, multiple large studies have yet to identify a causal gene ¹⁷⁾.

Mutations involving the SLITRK1 gene have been identified in a small number of people with Tourette syndrome ¹⁸⁾. The SLITRK1 gene provides instructions for making a protein that is active in the brain. The SLITRK1 protein probably plays a role in the development of nerve cells, including the growth of specialized extensions (axons and dendrites) that allow each nerve cell to communicate with nearby cells. It is unclear how mutations in the SLITRK1 gene can lead to Tourette syndrome ¹⁹⁾.

Most people with Tourette syndrome do not have a mutation in the SLITRK1 gene. Because mutations have been reported in so few people with this condition, the association of the SLITRK1 gene with this disorder has not been confirmed. Researchers suspect that changes in other genes, which have not been identified, are also associated with Tourette syndrome.

Tourette syndrome is inherited in an autosomal dominant disorder ²⁰⁾. Autosomal means that both males and females are affected, and dominant means that one copy of the gene is necessary to have the condition. This means that a parent with Tourette syndrome or a parent who has the gene for Tourette syndrome has a 50/50 chance, with each pregnancy, to pass the gene on to their child. Dominant disorders exhibit something known as incomplete penetrance, which means that not everyone with the gene will have symptoms of Tourette syndrome. In other words, if a parent passes the gene on to a child, the child may not have any symptoms of the Tourette syndrome. If a daughter inherits the gene, there is a 70 percent chance that she will have at least one of the signs of Tourette syndrome. On the other hand, if a son inherits the gene, there is a 90 percent chance that he will have at least one of the signs of Tourette syndrome.

Tourette syndrome is associated with a non-genetic cause in 10 to 15 percent of children. Some research has shown that Tourette syndrome is a genetically complex disorder that likely occurs as a result of the effects of multiple genes interacting with other factors in the environment. Scientists are studying other possible causes and environmental risk factors that might contribute to Tourette syndrome. Some studies have shown that the following factors might be associated with Tourette syndrome, but additional research is needed to better understand these associations ²¹⁾, ²²⁾, ²³⁾:

• Smoking during pregnancy.
• Pregnancy complications.
• Low birthweight.
• Carbon monoxide poisoning.
• Encephalitis. Researchers have found mixed results about whether certain children are more likely to develop tics following infections.

Risk factors for Tourette syndrome

Risk factors for Tourette syndrome include:

• Family history. Having a family history of Tourette syndrome or other tic disorders might increase the risk of developing Tourette syndrome.
• Sex. Males are about three to four times more likely than females to develop Tourette syndrome.

Tourette syndrome genetic changes

A variety of genetic and environmental factors likely play a role in causing Tourette syndrome. Most of these factors are unknown, and researchers are studying risk factors before and after birth that may contribute to this complex disorder. Scientists believe that tics may result from changes in brain chemicals (neurotransmitters) that are responsible for producing and controlling voluntary movements.

Mutations involving the SLITRK1 gene have been identified in a small number of people with Tourette syndrome. This gene provides instructions for making a protein that is active in the brain. The SLITRK1 protein probably plays a role in the development of nerve cells, including the growth of specialized extensions (axons and dendrites) that allow each nerve cell to communicate with nearby cells. It is unclear how mutations in the SLITRK1 gene can lead to this disorder.

Most people with Tourette syndrome do not have a mutation in the SLITRK1 gene. Because mutations have been reported in so few people with this condition, the association of the SLITRK1 gene with this disorder has not been confirmed. Researchers suspect that changes in other genes, which have not been identified, are also associated with Tourette syndrome.

Tourette syndrome hereditary

The inheritance pattern of Tourette syndrome is unclear. Evidence from twin and family studies suggests that Tourette syndrome is an inherited disorder. Although the features of this condition can cluster in families, many genetic and environmental factors are likely to be involved. Among family members of an affected person, it is difficult to predict who else may be at risk of developing the condition.

Tourette syndrome was previously thought to have an autosomal dominant pattern of inheritance, which suggests that one mutated copy of a gene in each cell would be sufficient to cause the condition. Several decades of research have shown that this is not the case. Almost all cases of Tourette syndrome probably result from a variety of genetic and environmental factors, not changes in a single gene.

Genetic studies also suggest that some forms of ADHD and obsessive–compulsive disorder (OCD) are genetically related to Tourette syndrome, but there is less evidence for a genetic relationship between Tourette syndrome and other neurobehavioral problems that commonly co-occur with Tourette syndrome. It is important for families to understand that genetic predisposition may not necessarily result in full-blown Tourette syndrome; instead, it may express itself as a milder tic disorder or as obsessive-compulsive behaviors. It is also possible that the gene-carrying offspring will not develop any Tourette syndrome symptoms.

The gender of the person also plays an important role in Tourette syndrome gene expression. At-risk males are more likely to have tics and at-risk females are more likely to have obsessive-compulsive symptoms.

Genetic counseling of individuals with Tourette syndrome should include a full review of all potentially hereditary conditions in the family.

Tourette syndrome signs and symptoms

Tourette syndrome symptoms are usually mild, but can sometimes be severe.

Tics are the main symptom of Tourette’s syndrome. Tics usually appear in childhood between the age of 5 and 10. The first symptoms often are motor tics that occur in the head and neck area. Tics usually are worse during times that are stressful or exciting. Tics tend to improve when a person is calm or focused on an activity.

Tics seen in Tourette syndrome change over time, and vary in frequency and complexity. Even though the symptoms might appear, disappear, and reappear, the tics are considered chronic in people with Tourette’s syndrome.

In most cases, tics decrease during adolescence and early adulthood, and sometimes disappear entirely. However, many people with Tourette syndrome experience tics into adulthood and, in some cases, tics can become worse during adulthood ²⁴⁾.

Tics are classified as:

• Simple tics. These sudden, brief and repetitive tics involve a limited number of muscle groups.
• Complex tics. These distinct, coordinated patterns of movements involve several muscle groups.

Tics also can involve movement (motor tics) or sounds (vocal tics). Motor tics usually begin before vocal tics do. But the spectrum of tics that people experience is diverse.

One set of symptoms is known as movement tics. People with movement tics can find themselves jerking their head, stretching their neck, stamping their feet, and twisting and bending. Some people also bite themselves or hurt themselves in other ways, or find it necessary to repeatedly touch other people and things.

Another set of symptoms is known as vocal tics. People with vocal tics might clear their throat, cough, sniff, click their tongue, grunt, yelp, bark or shout. Some also swear or repeat certain sounds or phrases.

People with Tourette’s syndrome might have both movement and vocal tics.

The following are the most common tics associated with Tourette syndrome. However, each child experiences symptoms differently. Tourette’s syndrome symptoms may include:

• involuntary, purposeless, motor movements (may involve different parts of the body, such as the face, neck, shoulders, trunk, or hands)
• head jerking
• squinting
• blinking
• shrugging
• grimacing
• nose-twitching
• any excessively repeated movements (i.e., foot tapping, leg jerking, scratching)

Some of the more complex tic behaviors associated with Tourette syndrome may appear purposeful, and may include the following:

• kissing
• pinching
• sticking out the tongue or lip-smacking
• touching behaviors
• making obscene gestures

In addition to some, or all, of the above symptoms, Tourette syndrome is also characterized by one or more vocal tics (meaningless sound), in order for a diagnosis to be made, including the following:

• grunting or moaning sounds
• barks
• tongue clicking
• sniffs
• hooting
• obscenities or swearing
• throat clearing, snorts, or coughs
• squeaking noises
• hissing
• spitting
• whistling
• gurgling
• echoing sounds or phrases repeatedly

Forty percent of children and adolescents who have Tourette syndrome also have attention problems, and 30 percent have academic difficulties. However, most have a normal intelligence and do not usually have primary learning disabilities. Twenty-five to 30 percent also experience symptoms of obsessive-compulsive disorder (OCD), which is an anxiety disorder in which a person has an unreasonable thought, fear, or activity (obsession) that he/she tries to manage through a ritualized activity (compulsion) to reduce the anxiety.

The symptoms of Tourette syndrome may resemble other conditions or medical problems. Always consult your child’s physician for a diagnosis.

Table 1. Common motor tics seen in Tourette syndrome

Table 2. Common vocal tics seen in Tourette syndrome

Someone with Tourette syndrome might be able to stop their tics for a short time, but this builds up tension until the tic ‘escapes’.

Tics worsen with stress and improve with relaxation or when the person is absorbed in a particular activity.

Tourette syndrome can be accompanied by other conditions, such as attention deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD).

Examples of movement/physical tics:

• blinking
• eye rolling
• grimacing
• shoulder shrugging
• jerking of the head or other limbs
• jumping
• twirling
• touching objects and other people

Examples of vocal tics:

• grunting
• throat clearing
• whistling
• coughing
• tongue clicking
• animal sounds
• saying random words and phrases
• repeating a sound, word or phrase
• swearing

Swearing is rare and affects only about 1 in 10 people with Tourette’s syndrome.

Tics aren’t usually harmful to a person’s overall health, but physical tics, such as jerking of the head, can be painful.

Tics can be worse on some days than others.

They may be worse during periods of:

• stress
• anxiety
• tiredness

People with Tourette’s syndrome can have behavioral problems, such as:

• antisocial behavior
• flying into sudden rages
• inappropriate behavior

Children with Tourette’s syndrome may be at risk of bullying because their tics might single them out.

Premonitory sensations

Most people with Tourette’s syndrome experience a strong urge before a tic, which has been compared to the feeling you get before needing to itch or sneeze.

These feelings are known as premonitory sensations. Premonitory sensations are only relieved after the tic has been carried out.

Examples of premonitory sensations include:

• a burning feeling in the eyes before blinking
• a dry or sore throat before grunting
• an itchy joint or muscle before jerking.

Co-occurring conditions

Tourette syndrome often occurs with other related conditions also called co-occurring conditions. These conditions can include attention-deficit/hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), and other behavioral or conduct problems. People with Tourette syndrome and related conditions can be at higher risk for learning, behavioral, and social problems. Most children who are diagnosed with Tourette syndrome also have another mental health, behavioral, or developmental condition ²⁵⁾.

The symptoms of other disorders can complicate the diagnosis and treatment of Tourette syndrome and create extra challenges for people with Tourette syndrome and their families, educators, and health professionals.

Conditions that commonly occur with Tourette syndrome include:

• Anxiety. There are many different types of anxiety disorders with many different causes and symptoms. These include generalized anxiety disorder (GAD), obsessive-compulsive disorder (OCD), panic disorder, post-traumatic stress disorder (PTSD), separation anxiety, and different types of phobias. Separation anxiety is most common among young children. These children feel very worried when they are apart from their parents.
• Depression. Everyone feels worried, anxious, sad, or stressed from time to time. However, if these feelings do not go away and they interfere with your daily life (for example, keeping a child home from school or other activities, or keeping an adult from working or attending social activities), a person might have depression. Having either a depressed mood or a loss of interest or pleasure for at least 2 weeks might mean that someone has depression. Depression can be treated with counseling and medication. Children and teens with depression might be irritable instead of sad. To be diagnosed with depression, other symptoms also must be present, such as:
  • Changes in eating habits or weight gain or loss.
  • Changes in sleep habits.
  • Changes in activity level (others notice increased activity or that the person has slowed down).
  • Less energy.
  • Feelings of worthlessness or guilt.
  • Difficulty thinking, concentrating, or making decisions.
  • Repeated thoughts of death.
  • Thoughts or plans about suicide, or a suicide attempt.
• Attention-deficit/hyperactivity disorder (ADHD). Attention-Deficit and Hyperactivity Disorder (ADHD) is a common co-occurring condition among children with Tourette syndrome ²⁶⁾. Children with ADHD have trouble paying attention and controlling impulsive behaviors. They might act without thinking about what the result will be and, in some cases, they are also overly active. It is normal for children to have trouble focusing and behaving at one time or another. However, for children with ADHD symptoms can continue, can be severe, and cause difficulty at school, at home, or with friends.
• Autism spectrum disorder
• Behavioral problems, such as oppositional defiant disorder (ODD) or conduct disorder (CD). Behavior problems can be highly disruptive for the child and others in the child’s life. It is important to get a diagnosis and treatment plan from a mental health professional as soon as possible. Effective treatments for disruptive behaviors include behavior therapy training for parents.
  • Children with oppositional defiant disorder (ODD) show negative, defiant and hostile behaviors toward adults or authority figures. Oppositional defiant disorder (ODD) usually starts before a child is 8 years of age, but no later than early adolescence. Children with oppositional defiant disorder (ODD) might show symptoms most often with people they know well, such as family members or a regular care provider. The behavior problems associated with oppositional defiant disorder (ODD) are mores severe or persistent than what might be expected for the child’s age, and result in major problems in school, at home, or with peers. Examples of oppositional defiant disorder (ODD) behaviors include:
    • Losing one’s temper a lot.
    • Arguing with adults or refusing to comply with adults’ rules or requests.
    • Getting angry or being resentful or vindictive often.
    • Annoying others on purpose or easily becoming annoyed with others.
    • Blaming other people often for one’s own mistakes or misbehavior.
  • Children with conduct disorder (CD) act aggressive toward others and break rules, laws, and social norms. They might have more injuries and difficulty with friends. In addition, the symptoms of conduct disorder happen in more than one area in the child’s life (for example, at home, in the community, and at school).
• Rage. Some people with Tourette syndrome have anger that is out of control, or episodes of “rage.” Rage that happens repeatedly and is disproportionate to the situation that triggers it may be diagnosed as a mood disorder, like intermittent explosive disorder. Symptoms might include extreme verbal or physical aggression. Examples of verbal aggression include extreme yelling, screaming, and cursing. Examples of physical aggression include extreme shoving, kicking, hitting, biting, and throwing objects. Rage symptoms are more likely to occur among those with other behavioral disorders such as attention-deficit and hyperactivity disorder (ADHD), oppositional defiant disorder (ODD), or conduct disorder (CD). Among people with Tourette syndrome, symptoms of rage are more likely to occur at home than outside the home. Treatment can include behavior therapy, learning how to relax, and social skills training. Some of these methods will help individuals and families better understand what can cause the symptoms of rage, how to avoid encouraging these behaviors, and how to use appropriate discipline for these behaviors. In addition, treating other behavioral disorders that the person might have, such as attention-deficit and hyperactivity disorder (ADHD), oppositional defiant disorder (ODD), or conduct disorder (CD) can help to reduce symptoms of rage.
• Developmental delays or intellectual disabilities
• Learning disorders
• Obsessive-compulsive behaviors. People with obsessive-compulsive behaviors have unwanted thoughts (obsessions) that they feel a need to respond to (compulsions). Examples of obsessive-compulsive behaviors are having to think about, say, or do something over and over. More than third of people with Tourette syndrome have obsessive-compulsive disorder (OCD) ²⁷⁾, ²⁸⁾, ²⁹⁾. Sometimes it is difficult to tell the difference between complex tics that a child with Tourette syndrome may have and obsessive-compulsive behaviors.
• Speech or language disorders

A Centers for Disease Control and Prevention (CDC) study ³⁰⁾ showed that the rates of asthma and hearing or vision problems were similar to children without Tourette syndrome, but bone, joint, or muscle problems as well as brain injury or concussion were higher for children with Tourette syndrome. Children with Tourette syndrome were also less likely to receive effective coordination of care or have a medical home, which means a primary care setting where a team of providers provides health care and preventive services.

Because co-occurring conditions are so common among people with Tourette syndrome, it is important for doctors to assess every child with Tourette syndrome for other conditions and problems.

Tourette syndrome complications

People with Tourette syndrome often lead healthy, active lives. However, Tourette syndrome frequently involves behavioral and social challenges that can harm your self-image.

Conditions often associated with Tourette syndrome include:

• Attention-deficit/hyperactivity disorder (ADHD)
• Obsessive-compulsive disorder (OCD)
• Autism spectrum disorder
• Learning disabilities
• Sleep disorders
• Depression
• Anxiety disorders
• Pain related to tics, especially headache
• Anger-management problems.

Educational concerns

As a group, people with Tourette syndrome have levels of intelligence similar to those of people without Tourette syndrome. However, people with Tourette syndrome might be more likely to have learning differences, a learning disability, or a developmental delay that affects their ability to learn.

Many people with Tourette syndrome have problems with writing, organizing, and paying attention. People with Tourette syndrome might have problems processing what they hear or see. This can affect the person’s ability to learn by listening to or watching a teacher. Or, the person might have problems with their other senses (such as how things feel, smell, taste, and movement) that affects learning and behavior. Children with Tourette syndrome might have trouble with social skills that affect their ability to interact with others.

As a result of these challenges, children with Tourette syndrome might need extra help in school. Many times, these concerns can be addressed with accommodations and behavioral interventions (for example, help with social skills).

Accommodations can include things such as providing a different testing location or extra testing time, providing tips on how to be more organized, giving the child less homework, or letting the child use a computer to take notes in class. Children also might need behavioral interventions, therapy, or they may need to learn strategies to help with stress, paying attention, or other symptoms.

Tourette syndrome diagnosis

Diagnosis of Tourette’s syndrome is made on clinical grounds. There’s no single test for Tourette’s syndrome. However certain tests, such as blood tests, EEG (scan of brain activity) and and MRI scan can help to exclude other conditions that could cause the symptoms.

You can be diagnosed with Tourette’s syndrome if you’ve had several tics for at least a year.

To be diagnosed with Tourette syndrome, a person must have:

• Two or more motor tics (for example, blinking or shrugging the shoulders) and at least one vocal tic (for example, humming, clearing the throat, or yelling out a word or phrase), although they might not always happen at the same time.
• Tics occur several times a day, nearly every day or intermittently, for more than a year.
• Tics begin before age 18.
• Tics aren’t caused by medications, other substances or another medical condition (for example, seizures, Huntington disease, or postviral encephalitis).
• Tics must change over time in location, frequency, type, complexity or severity.

A diagnosis of Tourette syndrome might be overlooked because the signs can mimic other conditions. Eye blinking might be initially associated with vision problems, or sniffling attributed to allergies.

Getting a firm diagnosis can help you and others understand your problems better, and help you access the right kind of treatment and support.

To get a diagnosis, your doctor may refer you to different specialists, such as a neurologist (a brain and nervous system specialist).

To rule out other causes of tics, your doctor might recommend:

• Blood tests
• Imaging studies such as an MRI

Tourette syndrome treatment

At present there are no curative treatments for Tourette syndrome and all treatment is symptomatic. Many people diagnosed with Tourette syndrome have only mild symptoms and do not require medication. If symptoms do interfere with daily life, medications are available to help control the symptoms, such as haloperidol and pimozide. Unfortunately, all medications have side effects and these need to be discussed with the doctor.

Other therapies, such as psychotherapy and relaxation techniques may also be useful.

Behavioral therapy

Behavioral therapy approaches are recommended as first line treatment ³¹⁾, ³²⁾. Behavioral therapy is a treatment that teaches people with Tourette syndrome ways to manage their tics. Behavioral therapy is not a cure for tics. However, it can help reduce the number of tics, the severity of tics, the impact of tics, or a combination of all of these. It is important to understand that even though behavioral therapies might help reduce the severity of tics, this does not mean that tics are just psychological or that anyone with tics should be able to control them. Behavioral therapy is usually provided by a psychologist or a specially trained therapist.

Two types of behavioral therapy have been shown to reduce tics.

Habit reversal training

Habit reversal is one of the most studied behavioral interventions for people with tics ³³⁾. Habit reversal training has two main parts: awareness training and competing response training. In the awareness training part, people identify each tic out loud. In the competing response part, people learn to do a new behavior that cannot happen at the same time as the tic. For example, if the person with Tourette syndrome has a tic that involves head rubbing, a new behavior might be for that person to place their hands on their knees, or to cross their arms so that the head rubbing cannot take place.

Exposure with response prevention

This method trains you to better control your urge to tic. Techniques are used to recreate the urge to tic to train you to tolerate the feeling, without doing the tic, until the urge passes.

Comprehensive Behavioral Intervention for Tics (CBIT)

Comprehensive Behavioral Intervention for Tics (CBIT) is an evidence-based type of behavioral therapy for Tourette syndrome and chronic tic disorders. Comprehensive Behavioral Intervention for Tics (CBIT) has been shown to be effective at reducing tic symptoms and tic-related impairment among children and adults. Comprehensive Behavioral Intervention for Tics (CBIT) is a non-drug treatment consisting of three important components ³⁴⁾, ³⁵⁾:

1. Training the patient to be more aware of tics
2. Training patients to do competing behavior when they feel the urge to tic
3. Making changes to day to day activities in ways that can be helpful in reducing tics.

In Comprehensive Behavioral Intervention for Tics (CBIT), a therapist will work with a child (and their parents) or an adult with Tourette syndrome to better understand the types of tics the person is having and to understand the situations in which the tics are at their worst. The first step in CBIT is to teach the patient to become more aware of his or her tics and the urge to tic. Next, the patient is taught to perform a specific behavior that makes the tic more difficult to do, as soon as the tic or urge appears. This “competing response” helps to reduce, and in some cases, even eliminate the tic. For example, a youngster with a frequent throat clearing tic would be taught to engage in slow rhythmic breathing whenever he felt the urge to clear his throat. A competing response chosen for a head-shaking tic might be gently tensing the head or neck muscles. Consistent and repeated practice of a carefully chosen competing response done at the appropriate time is necessary for the treatment to be effective.

Changes to the surroundings may be made, if possible, and the person with Tourette syndrome will also learn to do a new behavior instead of the tic (habit reversal). For example, if a child with Tourette syndrome often has a certain tic during math class, the math teacher can be educated about Tourette syndrome, and perhaps the child’s seat can be changed so that the tics are not as visible. In addition, the child also can work with a psychologist to learn habit reversal techniques. This helps to decrease how often the tic occurs by doing a new behavior (like putting their hands on their knees when an urge to perform the tic happens). CBIT skills can be learned with practice, with the help of an experienced therapist, and with the support and encouragement of those close to the person with Tourette syndrome.

CBIT is a highly structured therapy that typically takes place in a therapist’s office on a weekly basis. The standard treatment is 8 sessions over 10 weeks, but can be longer or shorter depending on the needs of the patient and his or her family.

The final step of CBIT, the functional intervention, is based on the fact that certain situations or reactions to tics can make them worse than they might otherwise be. The goal of functional intervention is to identify these situations and have the patient and family attempt to change them so the tics aren’t made worse unnecessarily. For example, someone whose tics get worse when doing homework or before a presentation at work would be taught to manage their stress before and during these situations.

Tourette syndrome medication

Medications do not eliminate tics completely. However, they can help some people with Tourette syndrome in their everyday life. There is no one medication that is best for all people. Most medications prescribed for Tourette syndrome have not been approved by the U.S. Food and Drug Administration (FDA) for treating tics. Haloperidol (Haldol), pimozide (Orap), and aripiprazole (Abilify) are currently the only medications approved by the U.S. Food and Drug Administration (FDA) to treat tics.

Medications can be used to reduce severe or disruptive tics that might have led to problems in the past with family and friends, other students, or coworkers. Medications also can be used to reduce symptoms of related conditions, such as ADHD or OCD.

• Medications that block or lessen dopamine. Fluphenazine, haloperidol (Haldol) and pimozide (Orap) can help control tics. Possible side effects include weight gain and involuntary repetitive movements. Tetrabenazine (Xenazine) might be recommended, although it may cause severe depression.
• ADHD medications. Stimulants such as methylphenidate (Concerta, Ritalin, others) and medications containing dextroamphetamine (Adderall XR, Dexedrine, others) can help increase attention and concentration. Although these drugs are somewhat effective, they have also been reported to increase tics for some people with Tourette syndrome, making their use controversial.
• Botulinum (Botox) injections. An injection into the affected muscle might help relieve a simple or vocal tic.
• Central adrenergic inhibitors. Medications such as clonidine (Catapres) and guanfacine (Tenex) — typically prescribed for high blood pressure — might help control behavioral symptoms such as impulse control problems and rage attacks. Side effects may include sleepiness.
• Antidepressants. Fluoxetine (Prozac, Sarafem, others) might help control symptoms of sadness, anxiety and OCD.
• Antiseizure medications. Recent studies suggest that some people with Tourette syndrome respond to topiramate (Topamax), which is used to treat epilepsy.

Medications affect each person differently. One person might do well with one medication, but not another. When deciding the best treatment, a doctor might try different medications and doses, and it may take time to find the treatment plan that works best. The doctor will want to find the medication and dose that have the best results and the fewest side effects. Doctors often start with small doses and slowly increase as needed.

As with all medications, those used to treat tics can have side effects. Side effects can include weight gain, stiff muscles, tiredness, restlessness, and social withdrawal. The side effects need to be considered carefully when deciding whether or not to use any medication to treat tics. In some cases, the side effects can be worse than the tics.

Even though medications often are used to treat the symptoms of Tourette syndrome, they might not be helpful for everyone. Two common reasons for not using medications to treat Tourette syndrome are unpleasant side effects and failure of the medications to work as well as expected.

Deep Brain Stimulation

For severe tics that don’t respond to other treatment, deep brain stimulation might help. Deep brain stimulation involves surgically implanting a pulse generator similar to a heart pacemaker into your chest wall. This is connected to one or two fine wires placed under the skin, and is inserted precisely into specific areas in your brain. A tiny electric current is produced by the pulse generator, which runs through the wire and stimulates the part of your brain affected by Tourette syndrome that control movement. However, deep brain stimulation is still in the early research stages and needs more research to determine if it’s a safe and effective treatment for Tourette syndrome.

Coping and support

Your self-esteem may suffer as a result of Tourette syndrome. You may be embarrassed about your tics and hesitate to engage in social activities, such as dating or going out in public. As a result, you’re at increased risk of depression and substance abuse.

To cope with Tourette syndrome:

• Remember that tics usually reach their peak in the early teens and improve as you get older.
• Reach out to others dealing with Tourette syndrome for information, coping tips and support.

Tourette syndrome outlook (prognosis)

Tourette syndrome is a lifelong condition and there is no cure for Tourette syndrome. 25% of patients may be symptom-free for periods of up to few years however only 8% of patients have complete and permanent remission. The condition in many individuals improves in the late teens and early 20s. As a result, some may actually become symptom-free or no longer need medication for tic suppression. Indefinite treatment is often required, however most patients can live an active normal life with a normal life expectancy. Although tic symptoms tend to decrease with age, it is possible that neurobehavioral disorders such as ADHD, OCD, depression, generalized anxiety, panic attacks, and mood swings can persist and cause impairment in adult life.

Recent study showed that one-third of Tourette syndrome will resolve completely, one-third will improve, and another third will continue without any reduction of their symptoms ³⁶⁾. Furthermore, tics appear to progressively decline during adolescence, as one study demonstrated a yearly decline in this demographic. In regards to the ‘third’ that experiences persistence of Tourette syndrome into adulthood, it has been postulated that coexistent psychopathologies are more likely present (e.g. ADHD, OCD) ³⁷⁾.