Tourette syndrome

Tourette syndrome: description

Twitching arms, grunts and snorting or even a multiple roar of swear words and obscenities such as “fat sow!” Or “Heil Hitler!” – People with Tourette syndrome can cause some confusion in their environment. Depending on how frequent and violent these tics are, they severely limit the quality of life of those affected.

Tourette syndrome in children

Tourette syndrome is not a mental disorder, but a neuropsychiatric disorder. The filter functions of the motor control fail. Tourette usually begins in childhood, rarely in adolescence. Especially younger children often go through a period of tics, which disappear after a few months on their own. Every tenth child increases symptoms – a Tourette syndrome develops. By definition, it is Tourette, if several motor tics (movements) occur together with at least one vocal tic (vocalizations) and these last at least one year.

In most people, the symptoms improve after puberty or disappear altogether. Others accompany the tics throughout their lives. Boys are affected four times as often as girls. The reasons are unknown.

The disease was first described in 1885 by French physicians Gille de la Tourette. He is the eponym for the “Gilles de la Tourette syndrome”.

Experts estimate that about one percent of people develop a Tourette syndrome – in Germany that would be about 800,000 people. However, only a small proportion is so severely affected that the disease is in need of treatment.

Classification in degrees of severity

The Tourette’s Syndrome Severity Scale (TSSS) helps determine the severity of the disease.

• Slight impairment: The tics do not affect the behavior at school or at work. Outsiders hardly notice the disturbance. The affected person perceives them as unproblematic.
• Moderate impairment: The tics are also noticeable to outsiders, so it always comes back to irritation. In addition, they complicate the execution of certain tasks in school or work.
• Serious impairment: The tics are so conspicuous that they massively disrupt social contacts and diminish performance. For those affected, they are a heavy burden.

Tourette syndrome: symptoms

A Tourette syndrome manifests itself in so-called tics. These can be involuntary movements or vocalizations. The term Tic comes from the French and means something like “twitching”. One distinguishes between motoric and vocal tics as well as simple and complex tics.

Motoric tics

easy motoric tics for example, winking, shrugging, turning your head or making faces.

Complex motor tics are touching objects or people, body twists or limbs. Obscene gestures may also appear (copropraxia). Sometimes it comes to self-injurious acts – those affected hit their heads against the wall, pinch or prick themselves with a pen.

Vocal tics

Simple vocal tics For example, sounds such as clearing your throat, squealing, grunting, sniffing, or snapping your tongue.

Complex vocal tics are words or sentences that are thrown out and have no logical connection with the situation. Often these are obscenities or swear words (coprolalia).

The range of tics is huge and individually very different. They change over time and new symptoms can be added. Some sufferers are even “inspired” by other Tourette patients – after an encounter they take over their tics.

The tics do not disappear during sleep and occur in all sleep stages. They are then weakened. In general, the patient has forgotten the occurrence of tics the next morning.

Convertible disease

A Tourette syndrome usually begins in childhood with simple motor tics, which can gradually increase. Later, vocalizations may be added. The tics often appear in series. Some sufferers only occasionally have tics – others are constantly forced to “gut”.

The tics can be suppressed for some time, but then come later the more violently to the outbreak. Some sufferers manage to master themselves while working or at school. At home your tics will give free rein. Others have virtually no control over the symptoms.

The tics occur several times a day, usually like an attack. This either happens almost every day, or they disappear for a while and then reappear in phases.

Emotional excitement such as pleasure, anger or anxiety aggravates the symptoms. The same applies to stress. If the patients are strongly focused on one thing, the tics decrease.

Sign of Tics

Sometimes the tics are triggered by sensorimotor signs, such as tingling or feelings of tension. These unpleasant sensations disappear when the tic is executed. As a rule, however, those affected also notice the tic only when it appears.

Other disorders

The vast majority of patients with Tourette syndrome develop more disorders. This includes:

• Attention Deficit Hyperactivity Disorder (ADHD)
• obsessive-compulsive disorder
• sleep disorders
• depressions
• anxiety disorders
• Social phobias

Tourette syndrome: causes and risk factors

The causes of Tourettesyndroms are so far only partially explored. It is believed that Tourette’s cases are genetically predisposed for the most part. Thus, the risk of Tourette is ten to a hundred times higher for children whose parents have the syndrome than for children without Tourette’s syndrome. For it to develop, additional triggers in the environment must be added. These include, for example, negative factors during pregnancy and childbirth, such as smoking and psychosocial stress during pregnancy, prematurity and lack of oxygen at birth. In addition, bacterial infections with group A streptococci are also considered to be possible triggers of Tourette’s syndrome.

Disturbed messenger metabolism

The emergence of Tourette’s syndrome is attributed to a disruption in the messenger metabolism of the brain. In particular, the neurotransmitter dopamine is in the focus of research: Dopamine is important in the brain for the forwarding of information. Among other things, studies have shown that the number of dopamine receptors in the brain of patients with Tourette’s syndrome is increased. But also a disturbed serotonin, norepinephrine, glutamine and opioid household as well as the interactions between these substances seem to play a role.

The disorders manifest themselves mainly in the so-called basal ganglia. These brain areas are located in the deeper structures of both halves of the brain and perform a kind of filtering function. They regulate which impulses a person translates into actions and which ones do not.

Tourette syndrome: examinations and diagnosis

Tourette’s syndrome is often diagnosed years after the onset of the first symptoms. Since the illness provides for misunderstandings and annoys the people, that is problematic. The children may be considered naughty and obstinate, the parents are worried because their education does not seem to be working properly. In such cases, the diagnosis is a relief for all concerned.

Some people with Tourette can control their tics for hours, so the doctor can not see them for themselves. The diagnosis of Tourette’s syndrome is therefore often based on the observation and description of the tics. Younger children often do not notice them themselves. Then it is the worried parents who tell the doctor about their symptoms.

Important questions are:

• How do the tics express themselves?
• Where, how often and how strongly do they appear?
• Does stress have a reinforcing effect on Tourette syndrome?
• Can the symptoms be suppressed?
• Are you announcing yourself by a kind of anticipation?
• At what age did the tics appear for the first time?
• Are the symptoms changing in terms of type, strength and frequency?
• Has there been any cases of Tourette syndrome within the family?

For the diagnosis Tourette’s syndrome, the tics must have existed for at least a year and manifested before the age of 18 years. There must have been various tic forms, including several motoric and at least one vocal tic.

For the Tourette syndrome, there are no laboratory tests or neurological and psychiatric examinations, with the help of which the diagnosis can be stopped. Therefore, examinations are mainly used to rule out other causes of tics or tic-like symptoms. That can be:

• Side effects of drugs (for example, neuroleptics)
• Brain tumors
• epilepsy
• Inflammation of the brain (encephalitis)
• Chorea (various dysfunction of the basal ganglia, which express themselves in involuntary movements)
• Ballismus (neurological disorder in which the patients perform abruptly throwing, throwing motions)
• Myoclonus (involuntary, sudden short muscle twitches of different origin)
• streptococcal infections

In the investigation, an electroencephalography (EEG) and a blood test can help. The latter is used to detect possible streptococcal infections and is particularly useful if the child had a otitis or scarlet fever.

Tourette syndrome: treatment

A Tourette syndrome is currently not curable. Existing therapies may improve the symptoms but have no influence on the course of the disease. Nevertheless, there are a number of offers that make life easier with a Tourette syndrome. Which option is ultimately chosen depends not only on the severity of the symptoms, but also on how strong the psychosocial burden is on the patient. So some sufferers with relatively pronounced tics feel little disturbed by these, while others already find it easier to create easier tics.

At the beginning of therapy there is always one psychoeducational counseling, In this context, patients are comprehensively informed about the disease, which already relieves many of them.

With moderate complaints helps in many cases one behavior therapyto get a grip on the tics. In severe cases can drugs help. There is a whole range of active ingredients available – but they often have serious side effects. These range from tiredness, dizziness and weight gain to a disturbed sexual function. Even with medication, the tics do not disappear completely. Relaistic is a reduction of up to 50 percent.

Even if medications fail, there is a chance to use one of the tics Brain pacemaker to tame.

It is crucial to treat not only the Tourette syndrome but also concomitant diseases such as ADHD, obsessive-compulsive disorder and sleep disorders. Frequently, the tics improve as well.

Psychoeducational counseling

As part of psychoeducational counseling, patients and their parents are informed about the background and prognosis of Tourette’s syndrome. Sometimes this is so relieving that they can tolerate the tics better. If the feeling of stress decreases, so does the stress that the disease brings. In this case, the development of the disease is only observed in order to be able to take further measures in the event of aggravation.

Behavioral treatment

As part of a behavioral therapy, patients learn to control their tics better. This has proven to be particularly effective Habit Reversal Training (HRT) proved. It is based on the idea that problematic behavioral problems sometimes occur unconsciously and eventually run automatically through constant repetition. In HRT, patients train their self-awareness and learn to break the automated behavioral chains through alternative actions.

Also effective seems a combination of exposure treatment and Response prevention, which is used mainly for the treatment of obsessive-compulsive disorder. Patients who notice the tic as a sensation, such as tingling or tense, learn that it does not necessarily have to be followed by a tic. According to first studies, both techniques lead to a tic reduction of 30 to 35 percent.

In addition, the psychological consequences of the disease can be absorbed by behavioral therapy. These include damaged self-esteem, insecurity in dealing with others, social phobias, anxiety disorders and depression.

Learning a relaxation technique can complement the behavioral therapy. With their help can reduce stress, which would otherwise increase the symptoms.

drugs

Medications for the treatment of Tourette syndrome have side effects – some serious. If the patient suffers greatly from his tics, they should still be used. This is necessary, for example if:

• The patient is suffering from pain due to tics (eg neck, back pain) or self-injured.
• The patient is socially marginalized, teased or bullied due to his tics. This is especially the case with vocal tics and strong motor tics.
• The patient has emotional problems such as anxiety, depression, social phobias or low self-esteem due to his illness.
• The patient has difficulties due to the symptoms to perform certain actions, fall asleep or even disturbed in his communication skills.

Most drugs used to treat Tourette’s syndrome target dopamine metabolism in the brain. The so-called Dopamine receptor antagonists dock to the various dopamine receptors and block them for the brain messenger. These include, above all, the various representatives of antipsychotic drugs (neuroleptics). They are considered the first-choice drugs for the treatment of Tourette’s syndrome. For therapy, the dose is slowly increased until a positive effect unfolds.

Classic neuroleptics: Haloperidol is the only active substance that is expressly approved in Germany for the treatment of Tourette’s syndrome. He helps around 70 percent of patients. Because of its side effects, it is now used in this country mainly when other drugs fail. This also applies to pimozide, which belongs to the same class of drugs. The unwanted concomitant symptoms include fatigue, weight gain and a disturbed sexual function.

Atypical neuroleptics: These agents may also reduce Tourette’s symptoms. Risperidone, for example, reduces tics by 41 to 62 percent. It also reduces aggressive behaviors that some Tourette patients develop. Side effects such as weight gain, prolactin elevation and sexual dysfunction are also problematic here. Another atypical antipsychotic prescribed in Tourette is aripiprazole.

benzamides: Benzamides such as tiapride and sulpiride inhibit the so-called D2 receptors in the brain. Although they help well, they are associated with side effects such as fatigue, dizziness, increased appetite and weight, hyperprolactinemia and sexual dysfunction. Tiapride is widely used in children because it does not affect their mental development and performance. Sulpiride is mainly prescribed for adults.

tetrabenazine Empties the dopamine storage in the brain. Initial studies indicate that it can reduce tics. However, side effects such as fatigue and depression may occur more frequently, so it is preferred to use other medicines.

Noradrenergic active substances: Clonidine, guanfacine and atomoxetine are mainly used in children with concomitant ADHD. Although they do not work as well as neuroleptics, but help against both disorders. Side effects include a dry mouth, headache, irritability and sleep disorders.

Dopamine agonists: Dopamine agonists such as talipexole have been used in only a few cases to treat Tourette. The effectiveness reports are inconsistent.

Nicotine: Nicotine, administered for example in the form of nicotine gums or patches, may possibly enhance the effect of neuroleptics in Tourette’s patients. It also increases the ability to concentrate. In fact, in isolated cases, smoking cessation has been shown to increase the symptoms of Tourette’s patients.

Botulinum toxin (Botox): Botox injections can help with tics on the face and neck. There are also reports that Botox improves vocal tics.

Cannabis: Some patients report that the use of cannabis relieves their symptoms. The effect is not proven. Recently, an application for treatment with a cannabis extract or herb has been submitted to the Federal Institute for Drugs and Medical Devices (BfArM).

Operations: Deep brain stimulation

For adult patients whose quality of life is severely limited by Tourette’s syndrome and who are not adequately helped by other therapies, deep brain stimulation is an option. For this, a brain pacemaker is planted underneath the abdominal skin, which electronically stimulates the brain via electrodes.

In other diseases, especially Parkinson’s, the procedure is already relatively common. With the Tourette syndrome, the case numbers and thus the experience are still relatively small. In particular, it is unclear which brain region needs to be stimulated in which patient. The success of the treatment is therefore very different: in some patients the procedure causes the symptoms to disappear almost completely. Others do not feel any effect.

Disease course and prognosis

A Tourette syndrome manifests in childhood and adolescence – usually between the fourth and eighth year of life. Usually, the disease starts with simple motor tics, later with vocal tics and symptoms become more complex. For most sufferers, the tics are constantly changing. In addition, worse phases alternate with lighter ones. For the majority of patients, the time between the age of eighth and twelfth is particularly difficult.

Generally the prognosis is favorable. In two-thirds of children, symptoms improve significantly over time or even disappear altogether. From the age of 18, most tics have dropped so much that they do not bother them anymore.

For the remaining third, the forecast is less favorable. In some of them, the symptoms become even more pronounced in adulthood. The loss of quality of life is especially great for them.

Living with Tourette syndrome

For the environment, the behavior of people with a Tourette syndrome is difficult to understand and often disturbing. Many find it difficult to accept that those affected are largely at the mercy of their tics. They are hostile and aggressive – especially on insults or obscene gestures. Of course, this is especially true when the patients are among strangers.

For some of those affected, these misunderstandings and environmental rejection are understandably angril, and they are reluctant to go among people. Also practicing certain occupations, especially those with many social contacts, is difficult for people with heavy touring.

Positive aspects of Tourette

People with Tourette syndrome are less controlled than others. That can also have advantages. For example, they are often very responsive. This is a big advantage in many sports. A lot of the people with Tourette syndrome also suffers from an attention deficit disorder (ADS). These people are very creative. The thoughts flow less controlled, which can be early, that easier new and unusual ideas arise.