Tourette's syndrome: what kind of disease is it, what causes it, symptoms, treatment, prognosis

What is Tourette's Syndrome?

Tourette's syndrome (Tourette's disease) is a condition that affects the brain and nervous system (neurological condition) and is characterized by involuntary, random vocal and motor nervous tics.

The disorder usually occurs during childhood.

The disease is named after the French physician Georges Gilles de la Tourette, who first described the condition and its symptoms back in the 19th century.

Nervous tics

Ticks can be:

• vocal (making sounds) - for example, wheezing, coughing, or shouting out words;
• physical (motor) - such as head twitching or jumping up and down.

Ticks can also be:

• simple, involving the movement of only one muscle or making one sound;
• complex, involving a series of physical movements or shouting out long phrases.

Most people diagnosed with Tourette's syndrome have a combination of physical and vocal tics that can be simple or complex.

Nervous tics are usually not a serious threat to a person's physical health, although physical tics, such as twitching of the head, can often be painful. However, children and adults with Tourette's disease can experience accompanying problems such as social isolation, embarrassment, and low self-esteem.

Some people with Tourette's syndrome only occasionally experience tics and do not need treatment. When tics are more common, there are several medications that can help control them quite effectively. A type of psychotherapy known as behavioral therapy can also be effective for people with Tourette's disease.

How common is the disease?

Tourette's syndrome is far more common than many people think, as it affects about 1 in every 100 people.

Symptoms usually begin around the age of seven and become most pronounced during adolescence.

Boys are more likely to suffer from Tourette's syndrome than girls. It is unclear why this is so.

Causes of Tourette's Syndrome

The cause of Tourette's syndrome is unknown. However, the condition is thought to be related to problems with the basal ganglia.

Basal ganglia

The basal ganglia are a group of specialized brain cells located deep within the brain. They help regulate physical body movements.

Recent research suggests that the basal ganglia may play a more fundamental role in higher brain functions such as motivation and decision making.

For example, on a very simple level, if you suddenly decide that the TV program you are watching is boring, your basal ganglion will prompt your body to pick up the remote and change the channel.

It seems that in people with Tourette's, tics occur when there is a temporary disruption within the basal ganglia and decision-making becomes erratic. The victim suddenly develops an unconscious urge or motivation to perform an action (tic) that the conscious mind considers undesirable and inexplicable.

What exactly is wrong with the basal ganglia is still a matter of controversy. One of the theories is that it may be caused by an excessive amount of a natural chemical called dopamine (dopamine), which can have powerful effects on the brain.

Alternatively, dopamine levels may be normal, but people with Tourette's syndrome are especially sensitive to its effects.

Brain studies have also shown that the structure of the basal ganglia differs in people with Tourette's syndrome. Whether these changes are a result of an imbalance or sensitivity to dopamine or a cause is unclear.

Main reasons

What causes Tourette's syndrome is unclear. There are several theories that are outlined below.

Genetics

Genetics seems to play an important role in some cases of Tourette's, as it occurs in families.

Further evidence suggests that if one identical twin develops Tourette's syndrome, there is a chance that the other twin will develop the condition as well.

It is possible that a genetic mutation disrupts normal brain development, causing the symptoms of Tourette's syndrome. A genetic mutation is when the instructions contained in all living cells (genes) are somehow intermixed.

Childhood infection

Another theory is that Tourette's syndrome may be related to childhood infection with strep bacteria (a type of bacteria that commonly causes throat infections).

In an attempt to fight off infection, the immune system produces antibodies. These antibodies can then interact with brain tissue, affecting normal brain development.

Some children have developed symptoms of Tourette's syndrome as well as symptoms of obsessive-compulsive disorder (OCD) after a throat infection.

Some doctors have suggested that it might be a separate condition in itself and have called it “childhood autoimmune neuropsychiatric disorders associated with streptococcal infections ", or PANDAS for brevity.

The PANDAS concept is controversial as research results are conflicting. It may be that PANDAS is responsible for only a small minority of cases of Tourette's disease. Further research is needed to clarify the problem.

Symptoms of Tourette's Syndrome

Having tics does not mean your child has Tourette's, as many babies have tics for several months before they grow out of them.

Tics caused by Tourette's syndrome can be:

• physical, such as head twitching or blinking;
• vocal (making sounds), such as shouting out words.

Ticks can be further classified as:

• simple, involving the movement of only one muscle or making one sound;
• complex, involving a series of physical movements or shouting out long phrases.

Most children diagnosed with Tourette's syndrome have a combination of physical and auditory tics that can be simple or complex.

Simple physical tics

Examples of simple physical ticks include:

• blinking eyes;
• head twitching;
• twitching of the nose;
• teeth grinding;
• rolling eyes;
• twisting of the neck;
• twitching shoulders.

Simple sound tics

Examples of simple audible ticks include:

• grunt;
• screams;
• cough;
• wheezing;
• barking;
• hiss;
• sniffing.

Difficult physical tics

Examples of complex physical ticks include:

• head shaking;
• hitting or kicking objects;
• jumping;
• touching yourself or others;
• copying the movements of other people - the medical term "echopraxia";
• making obscene gestures, such as showing someone the "middle finger" - known as copropraxia.

Complex sound tics

Examples of complex audio ticks include:

• repetition of phrases of other people - echolalia;
• repeating the same phrase over and over again is palilalia;
• Shouting swear words or inappropriate words or phrases is coprolalia.

While many people associate yelling swearing with Tourette's, it is actually a relatively rare symptom and affects only a minority of people with the syndrome.

Preliminary sensations

Most people with Tourette's syndrome find they experience uncomfortable or unusual physical sensations before they have a tick appears, and then this feeling is relieved only after they perform the tick - just like the itching, which can only be relieved by scratching.

These types of feelings are known as preliminary sensations.

Examples of pre-sensations include:

• a burning sensation in the eyes, a feeling that it can only be removed by blinking;
• muscle tension that can only be relieved by twitching or stretching the muscle;
• dry and sore throat that can only be relieved by coughing or clearing the throat;
• itching of a joint or limb, which can only be relieved by twitching the joint or limb.

Tick ​​triggers

If a child has Tourette's syndrome, you will likely find that their tics follow an established pattern. Tics are usually worse during periods:

• anxiety;
• stress;
• tiredness;
• illness;
• nervous excitement.

On the other hand, tics tend to be calmer when the child is engaging in enjoyable activities that include high levels of concentration, such as:

• reads an interesting book;
• is engaged in sports;
• plays a computer game.

You may find that your child can have some degree of control over their tics when they are in a place where they will be most noticeable, such as in a classroom or lecture hall. Controlling ticks, however, can be a difficult and tedious process over long periods of time.

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

When to Seek Medical Help

It is always recommended to seek medical advice if you or your child develop tics.

Many babies have tics for several months before they grow out of them, so the appearance of tics does not automatically mean that the child has Tourette's syndrome.

However, symptoms such as nervous tics warrant further investigation and possibly referral to a doctor who specializes in conditions that affect the brain and nervous system (neurologist).

Brain plasticity

The human brain has a high degree of what neurologists call "plasticity». This means that different networks and pathways between brain cells do not stand still, but can change over time and adapt to new networks and pathways.

The reason many people grow out of Tourette's is believed to be because their the brain effectively "rearranges" itself to compensate for the problems with basal ganglia.

Once these changes are completed, the symptoms will either improve dramatically or disappear completely.

Diagnosing Tourette's Syndrome

The first step in confirming the diagnosis of Tourette's syndrome is to rule out other possible causes of the child's symptoms..

They include:

• allergies if they make sniffing and coughing sounds
• vision problems if they blink more than usual

It is also necessary to rule out other diseases that cause tic-like behaviors, such as:

• autism spectrum disorder - a developmental disorder causing problems with social interaction, learning and behavior;
• dystonia - a condition causing involuntary muscle spasms;
• Huntington's diseaseIs a genetic disorder that causes a variety of symptoms, including muscle twitching.

To rule out this, the child may be referred to a number of specialists, such as:

• neurologist - a physician specializing in the treatment of conditions affecting the brain and nervous system;
• psychiatrist - a physician specializing in the treatment of mental illness;
• educational psychologist - a healthcare professional who works with children who have learning, developmental or behavioral problems.

To rule out other possible causes of symptoms, the child may be referred for an examination, such as an eye exam or an allergy skin test (prick test). The pric test is used to detect allergies in a person. Small amounts of potentially allergic substances, such as pollen, are injected under the skin to see if there is a reaction.

Brain scans can also be used to check for any brain abnormalities. and the nervous system, which may indicate a neurologic cause of symptoms other than the syndrome Tourette. Visual examinations that can be used include:

• computed tomography (CT) - a series of X-rays to create a detailed three-dimensional image of the brain;
• magnetic resonance imaging (MRI) - where strong fluctuating magnetic fields are used to produce a detailed image of the inside of the brain.

Confirmation of the diagnosis

There is currently no test that can be used to diagnose Tourette's syndrome.

Diagnosis can only be made by evaluating the symptoms to determine if they are consistent with the usual pattern associated with the disease.

A confident diagnosis of Tourette's syndrome can usually be made if:

1. Symptoms are not caused by other medical conditions or medications.
2. The child began to experience tics before the age of 18.
3. The child had multiple physical tics and at least one audible tic.
4. A child's tics occur many times throughout the day, almost every day.
5. Nervous tics last at least a year.

Treatment for Tourette's syndrome

There are various treatment options for Tourette's syndrome, which are described below.

Treatment plan

Your treatment plan may include one or more of the following:

1. Treatment that does not include medication, such as relaxation therapy or behavioral therapy (sometimes called non-pharmacological treatment).
2. Medication. There are three types of drugs: alpha2-adrenergic agonists, muscle relaxants, and antipsychotics.
3. Surgery. Several recently developed surgical techniques can be used to treat some isolated cases of Tourette's syndrome.

If a child's tics are relatively mild and rare, then they may only need behavioral therapy.

If their tics are more severe and interfere with their daily activities, then they are likely to benefit from a combination of therapy and medication.

Surgery is usually only recommended as a “treatment of last resort” for patients with very severe symptoms who do not respond to other treatments.

The physician in charge of treatment and care is usually a neurologist (specialist in treating conditions affecting the nervous system), - will recommend what they think is the best treatment option, but the final decision will be After you.

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

Behavioral therapy

Behavioral therapy is a widely used non-pharmacological treatment for Tourette's syndrome.

Behavioral therapy is a type of psychotherapy designed to change the patient's behavior patterns.

One type of behavioral therapy that has been shown to be successful in treating Tourette's syndrome is called habit change. Habit change is based on two main principles:

1. People with Tourette's are often unaware of their tics.
2. Tics are used to relieve discomfort (sensations that often occur before tics).

First stage - track the nature and frequency of tics and identify any sensations that cause them.

The next step is the search for an alternative, less noticeable way of getting rid of the sensations that arise during sleep, instead of tics. This is known as a competing response.

For example, a child may have an uncomfortable sensation in the throat that causes him to make a sound. Therefore, the next time the child feels an unpleasant sensation, he will be asked to take a series of deep breaths, and not cough or publish others. sounds to try and ease that sensation.

Habit withdrawal is often combined with relaxation therapy. Relaxation techniques such as deep breathing or visualization (thinking pleasant things to distract yourself) can help prevent stress and anxiety in the child. Stress and anxiety often make tics worse.

Drug treatment

Alpha2-adrenergic agonists

Alpha2-adrenergic agonists are generally recommended for the treatment of mild to moderate symptoms of Tourette's syndrome.

These types of drugs are thought to stabilize the levels of a chemical called norepinephrine inside the brain. This, in turn, is believed to reduce the likelihood of misfiring and triggering tics of the basal ganglia.

A widely used alpha2-adrenergic agonist in the treatment of Tourette's syndrome is called "Clonidine".

Common side effects of clonidine include:

• drowsiness;
• headaches;
• dizziness;
• fatigue;
• constipation;
• diarrhea;
• dry mouth.
• difficulty sleeping.

These side effects are usually minor and should improve as the child gets used to the medication.

Muscle relaxants

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

Baclofen and clonazepam Are two commonly used muscle relaxants that are used to treat Tourette's syndrome. The patient may feel drowsy and dizzy after taking it.

If you are an adult with Tourette's who is being treated with muscle relaxants, then you should not drive a car or use tools or machinery if you feel dizzy and drowsiness. It is also recommended to avoid drinking alcohol while taking muscle relaxants, as this can increase adverse reactions.

Antipsychotics

Antipsychotics are the most effective medication for preventing tics. However, they cause a wide range of side effects and are therefore only recommended if the child's symptoms are particularly severe or do not respond to other medications.

Antipsychotics block the effects of dopamine on the brain. Dopamine is one of the chemicals thought to be associated with tics.

There are two main types of antipsychotics:

1. typical antipsychotics, the first generation of antipsychotics that were developed during the 1950s;
2. atypical antipsychotics, a new generation of antipsychotics that were developed during the 1990s.

Atypical antipsychotics are generally recommended because they are less likely to cause side effects.

However, they are not suitable or effective for everyone.

Antipsychotics are administered orally (as a tablet) or by injection.

Both typical and atypical antipsychotics have side effects, although not everyone will experience them, and their severity will be different for everyone.

Side effects of typical antipsychotics include:

• drowsiness;
• dizziness;
• shiver;
• muscle twitching;
• cramping.

Side effects of both typical and atypical antipsychotics include:

• weight gain;
• blurred vision;
• constipation;
• dry mouth.

Adults with Tourette's who are treated with antipsychotics may also find that they have decreased sex drive (loss of libido).

If the side effects of the child are particularly unpleasant, see the doctor who treats the child to have a specialist suggest an alternative antipsychotic drug.

Surgery

Surgery is generally viewed as a last resort for people with severe Tourette's who have failed to respond to other treatments. Usually recommended for adults only.

The two types of surgery used to treat Tourette's syndrome are described below:

Limbic leukotomy

A technique known as limbic leukotomy can be used to treat Tourette's syndrome. The technique includes a neurosurgeon (a surgeon who specializes in operations of the brain and nervous system) using an electric current or pulse of radiation to burn a small part of the limbic systems.

Limbic system - a structure in the brain responsible for some of the most important functions of the brain, such as emotion, memory and behavior.

By burning a small part of the limbic system, the surgeon can often "remount" the brain and, in the process, eliminate conditions such as Tourette's syndrome in whole or in part.

Deep brain stimulation

For the treatment of Tourette's syndrome, a newer therapy is available, such as deep brain stimulation (DBS). Electrodes are permanently implanted in those parts of the brain known to be associated with Tourette's syndrome.

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

Initial DBS results were encouraging, with some early-stage patients experiencing significant tic reduction lasting more than 5 years.

Deep brain stimulation is most effective when combined with the behavioral therapy described above.

Complications of Tourette's syndrome

Complications of Tourette's syndrome can include some psychological and behavioral problems, as well as learning difficulties. However, Tourette's syndrome does not usually affect a person's intelligence.

Obsessive compulsive disorder

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

OCD is a long-term (chronic) mental health condition that is usually associated with obsessive thoughts and some compulsive behavior.

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

• constant checking of things - for example, patients check whether the windows or the door are locked;
• striving for order and symmetry - for example, their toys must be lined up on the shelf in one specific way, and if this order is violated, the child can become very upset;
• storage - storage of items of no real value, such as bus tickets or flyers;
• cleaning is a constant cleaning because they have an obsessive fear of disease and pollution: this often takes the form of forced hand washing.

Often, physical tics and compulsive behavior can combine, so the child can constantly pick up an object and lay it down or open and close the door repeatedly.

OCD is treated with a combination of medications such as selective serotonin reuptake inhibitors (SSRIs), antidepressants, and therapy such as cognitive behavioral therapy (CBT).

With treatment, most people experience an improvement in their symptoms, and some achieve a complete cure.

Attention Deficit Hyperactivity Disorder (ADHD)

Another common condition that affects children with Tourette's syndrome is attention deficit hyperactivity disorder (ADHD), which is thought to affect up to 70% of children with Tourette's syndrome.

ADHD is a behavioral condition that causes problems with tasks such as attention, concentration, impulse control, and the ability to plan ahead.

Children with Tourette's and ADHD tend to have great difficulty concentrating on specific tasks for extended periods of time and are often easily distracted.

Behavioral problems

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

• irritability;
• anxiety;
• antisocial behavior;
• sudden rage;
• inappropriate behavior towards others.

As a child grows older, this inappropriate behavior can often take the form of inappropriate sexual remarks or behavior in a sexually aggressive manner.

These types of problems often improve when the child begins treatment for Tourette's syndrome and their tics are better controlled.

Learning difficulties

Tourette's syndrome does not usually affect a person's intelligence, but it can cause learning difficulties, especially if the person also has attention deficit disorder with hyperactivity disorder (ADHD) or obsessive-compulsive disorder (OCD).

Many people with Tourette's have a harder time learning out of habit, such as when a child is learning to read. This is because the part of the brain that controls skill learning is the basal ganglia, which are most associated with Tourette.

Consequently, children with Tourette's syndrome may have difficulty learning skills and activities that other children acquire in daily life, for example, by adding or subtracting prime numbers, reading and letter.

Some children with Tourette's disease may require additional specialized educational support.

Forecast

Two-thirds of people will experience a marked improvement in their symptoms, usually about 10 years after their onset. Many of these people no longer need medication or therapy to control their tics.

For some people, Tourette's symptoms become much less unpleasant and frequent, while for others, they may disappear completely.

The remaining third of people with Tourette's will have symptoms for their entire lives, but symptoms tend to get milder as they get older. This means that their need for drugs and therapy may wear off over time.