Tourette syndrome

• Danger:

Synonyms in a broader sense

• Gilles de la Tourette syndrome
• Tourette's disease / disorder
• generalized tic disease with motor and vocal tics

Medical: Myospasia impulsiva

definition

As Tourette syndrome denotes a neurological-psychiatric disease caused by muscular (motor) and linguistic (vowels) Tics is characterized, but they do not have to occur simultaneously. Tourette syndrome is often associated with behavioral disorders.

Tics are simple or complex, sudden, short-lived, involuntary or semi-voluntary movements or noises and vocalizations.

Epidemiology

The frequency for the Tourette syndrome in the general population lies between 0,03% and 1,6%, there are also studies with the values ​​between 0,4% and 3,8% gives. This suggests a different frequency of the disease in different populations. Tourette's syndrome, for example, appears to be much less common among African-Americans and rarely occurs in sub-Saharan Africa. However, Tourette's syndrome can be found in all cultures, albeit with different frequencies. In general, however, it can be said that around 1% of all young people worldwide are affected.

In Germany it is 0,2% - 1,5% of the general population, with men three times more likely to be affected than women.

history

The disease was 1825 first time by Jean Itard, a French doctor and pedagogues (1774-1838), mentioned in the medical literature. He described the conspicuous behavior of the Marquise de Dampierrewho have had complex vowels since she was 7 years old Tics that included strange movements, strange noises and often obscene expressions. Because of this behavior, she had to withdraw from public life and died lonely at the age of 86.

The name Tourette syndrome goes to the French neurologist (see also neurology) George Gilles de la Tourette, who 60 years later published a study of the Marquise de Dampierre and eight other patients suffering from similar tics. The study was titled: "Étude sur une affection nerveuse caracterisée par l'incoordination motrice accompagnée d'écholalie et de coprolalie de la Neurologie, paris 9, 1885, 19-42 et 158-200 “Dr. Tourette described the nervous disease as the "Maledie des Tics.“

Also Mozart and André Malraux are said to have suffered from Tourette's syndrome.

causes

The cause of the Tourette syndrome is not clear. However, malfunctions in the systems of the Brain like that Basal gangliathat carry the messenger (Transmitter) Have dopamine. Transmitters are substances that serve to transmit signals in the brain and, in the case of Tourette's syndrome, are overly active. The thesis is supported by the fact that antagonists of dopamine (Dopamine antagonists) reduce tics, whereas substances that mimic the effects of dopamine (Dopamimetics) and thus increase the dopamine effect, as well as substances such as Amphetamines, Trigger tics. In addition, the number of docking points (Receptors) for dopamine (D2-receptor) the severity of the disease.

In addition, faults in the systems in which Serotonin is present as a messenger substance.

Tourette's syndrome is also believed to be hereditary (hereditary) Illness. In 60% of patients, tics can be found in family members, so there is a so-called "positive family history". The inheritance process is presumably dominant or semi-dominant, i.e. only one parent needs to have the diseased gene in order for their child to also receive it Tics or on that Tourette-Syndrome sick. A Tourette patient therefore inherits with a probability of 50% his diseased gene. However, the manifestation can be very different, so the disease does not have to have the full picture of Tourette's syndrome, but can also only contain slight tics. The expression depends, for example, on whether the diseased gene was inherited from the mother or the father (genomic imprinting). In general, it can be said that women are affected less often and less severely than men. The exact location of the affected gene has not yet been found.

Tics were also developed when people stopped taking so-called nerve depressants (Neuroleptics) and drugs against epilepsy (Anti-epileptic drugs) observed.

In recent years the possibility has been increasingly considered that it might be the Tourette syndrome could also be an autoimmune disease (see also: immune system). Autoimmune diseases are diseases that are caused by an excessive reaction of the immune system, so that the immune system attacks the own body. This could be the case as a result of an infection of the throat and pharynx or the middle ear with streptococci. Motor and vowels Tics and Obsessive-compulsive disorder closely related to a streptococcal infection are under the PANDASSyndrome (Pediatric A.utoimmune Neuropsychiatric D.isorders A.ssociated with S.treptococcal infections).

Symptoms

Symptoms are those already mentioned motor and vowels Tics. This can include: twitching of the neck and face, decreased control of impulses, forced throat clearing, repeated uttering of obscene and aggressive expressions (coprolalia), indecent movements such as masturbation movements (copropraxia), repeating sounds or words that have just been heard are (echolalia), repetition of coordinated movements just seen (echopraxia) and the repetition of syllables (palilalia). The motor tics can be so severe that normal voluntary movements of the hands are impossible. Around 10% of patients suffer from so-called restless leg syndrome, which causes involuntary movements of the legs.

There are also certain side effects of Tourette's syndrome, but they do not necessarily belong to the clinical picture. These include non-fluent speech, childhood hyperactivity disorder, attention deficit disorder, compulsive behaviors such as counting or touching, self-destructive behaviors such as deliberately hitting the head, or other abnormal behavior.

Jerks in the neck and face area also include twitching of the eyelids, but the causes are very diverse and cannot be caused exclusively by Tourette's syndrome:
Twitching eyelid - these are the causes

course

The first symptoms of Tourette's syndrome usually appear between the ages of 2 and 15 and rarely after the age of 20. Motor tics are the initial symptoms, around 50% develop complex motor tics, i.e. tics in which several muscle regions are involved, such as clapping. Echolalia occurs in up to 35% of cases and coprolalia in 60%. Many patients experience a complete relief of symptoms (Remission) or at least to a significant improvement. Often times, patients with Tourette's disorder also have obsessive-compulsive disorder or had attention and hyperactivity disorder as children.

Read more on the subject under: Jerks

diagnosis

So that a disease is classified as Tourette-Syndrome can be diagnosed, it must meet the following diagnostic criteria according to the Diagnostic and Statistical Manual of Mental Disorders (American Psychiatric Association 1987) fulfill:

1. Multiple motor and one or more vocal tics at a time during the course of the disease, but not necessarily simultaneously
2. Repeated occurrence of tics during the day, practically every day, or recurring over a period of more than a year
3. Regular change in the number, frequency and type of tics as well as the body region in which they occur and the changing course in the severity of the symptoms
4. Occurrence before the age of 21

Not necessarily for that Diagnosis Tourette-Syndromes are coprolalia, copropraxia, echolalia, echopraxia and palilalia, which are probably the most striking and remarkable symptoms for the layperson.

The diagnosis is made through questioning (anamnese) of the patient and observation of the symptoms over a longer period of time so that the severity of the disease can be determined. This is done with the help of questionnaires and estimation scales that have been specially developed for the reliable diagnosis of Tourette's syndrome. It is also important to assess the medical history of the patient himself and his family. However, there is no specific examination, neither laboratory nor imaging. However, a measurement of brain waves (electroencephalogram, EEG) and a method for the production of virtual sectional images (single-photon emission computed tomography, SPECT) of the brain to differentiate Tourette's syndrome from other diseases. In the advanced stages of the disease, SPECT shows reduced binding of dopamine to the D2 receptors.

If the cause is an autoimmune reaction, certain antibodies can be detected.

Differential diagnosis

The motor tics that are an integral part of Tourette's syndrome must be involuntary from rapid Muscle twitching (Myoclonia) and Movement disorders (Dystonia) can be distinguished. Tics can be suppressed for a certain period of time, but myoclonia cannot be suppressed and dystonia can only be suppressed to a certain extent. In addition, tics are accompanied by a previous discomfort that triggers the actual movement. This sensory component is the main difference to other movement disorders.

therapy

Genetic studies have shown a connection between the Tourette syndrome, chronic tics, and obsessive-compulsive disorder are proven. This close connection between the diseases is important in therapy because Tourette patients are affected by the psychiatric disorder (see also: Psychiatry online) are often more impaired than by motor or vocal tics. However, there are also patients who, over time, learn to deal with their tics themselves and thus need neither psychotherapeutic nor medicinal treatment. However, it is always important to inform the patient's social environment about the disease so that acceptance increases and the patient is prevented from becoming isolated. The therapy of Tourette's syndrome can only be done symptomatically, i.e. only the symptoms, i.e. the tics, are treated, the cause, however, is mostly unexplained and cannot be treated.

Behavioral therapy often makes sense to learn how to cope with tics in everyday life. They become weaker, for example, when concentrating on a thing or an action stress but stronger. Drug therapy is usually only used if the tics are so frightening for the environment that the patient is restricted too much, or in the case of aggressive tics that are directed against the patient or other people. The most effective tic-reducing drugs are Neuroleptics how Haloperidol, Pimozide and Fluphenazinewhose effect comes about by influencing dopamine receptors. However, it is important to weigh up the benefits of the therapy and the possible side effects of the medication. The use of neuroleptics leads to tiredness and decreased motivation, which is particularly problematic for school children. In addition, neuroleptics carry the risk of disrupting the Movement coordination (Dyskinesia), which is why they should only be prescribed in severe cases. Clonidine, Tiapride and Sulpiride are less full of side effects, but not as effective.

Danger:

Drugs used to treat hyperactivity or obsessive-compulsive disorder in children can lead to an increase in tics!

Rehabilitation / prognosis

The prognosis is the same for most child and adolescent patients Tourette syndrome pretty good. Many of the patients are tic-free by the end of their first or early second decade of life, so symptoms have completely subsided (Remission) or at least to a significant improvement. However, there may be a consolidation of side effects such as behavioral disorders or Learning problems come in adulthood. Over half of those affected feel more restricted by these behavioral problems than by the tics.

For the severely affected adult patients, new drugs are being developed, as well as a new method of deep brain stimulation, so that a higher quality of life can be ensured for the sick people.

Summary

The Tourette syndrome is a neurological-psychiatric disease caused by motor and vowels Tics is characterized and is usually accompanied by behavioral disorders. The diagnosis is made through detailed questioning (anamnese) and observation of the patient over a longer period of time using questionnaires and estimation scales. Therapy is symptomatic and often also psychotherapeutic. Drug therapy with neuroleptics is only recommended if the patient is suffering from extreme psychological stress, difficulties at school, work or family, or if aggressive tics occur that could injure the patient or people around him. Many of the child and adolescent patients experience a decrease in symptoms (Remission) from the age of 18.