Tourette syndrome

from Wikipedia, the free encyclopedia
Classification according to ICD-10
F95.2 Combined vocal and multiple motor tics [Tourette's syndrome]
ICD-10 online (WHO version 2019)
Gilles de la Tourette

The Gilles de la Tourette syndrome (short Tourette's syndrome ) is a congenital disease of the nervous system . The cause is often due to changes in the genetic make-up . The main features are involuntary movements ( tics , from French tic , `` nervous twitching '' ) and also tic-like phonetic or linguistic expressions. Simple motor tics can manifest themselves as blinking eyes, wrinkling the nose, throwing the head or making faces. Examples of simple vocal tics are making meaningless sounds, coughing, or mimicking animal noises. The category of complex tics in the motor area includes making faces and imitating the actions of others. Complex vocal tics are repeating words or throwing out obscene and aggressive expressions. Tourette's syndrome is counted among the central nervous movement disorders . Primary tic disorders can neither be cured nor treated causally. There are only soothing treatment approaches available.

The name refers to the French neurologist and psychiatrist Georges Gilles de la Tourette , who first described the disease in 1884/1885 at the suggestion of his teacher Jean Martin Charcot . However, Jean Marc Gaspard Itard reported on one of his patients as early as 1825 .

distribution

In children, the estimated prevalence is 0.3 to 0.9 percent. The incidence is significantly lower in adults. Differences in frequency in international comparison are attributed to cultural differences, since the symptoms themselves are similar everywhere and therefore point to common biological causes. It is diagnosed about three times more often in boys than in girls.

Signs

Tourette's syndrome has specific key symptoms and usually other abnormalities. The actual appearance varies from patient to patient. Tics are involuntary, rapid, usually suddenly shooting and sometimes very violent movements that can occur again and again in the same way, individually or in series. Loud, unwanted utterances such as exclamations or noises are also included.

The main symptoms are motor and vocal tics of various kinds, which often appear for the first time in elementary school age and are usually fully developed up to around the age of 14. An intensification can often be seen in puberty . In some patients, tics subside between the ages of 16 and 26, but the majority of people have a lifetime of trying to cope with them.

Simple motor tics can manifest themselves as blinking eyes, wrinkling the nose, throwing the head or making faces. Examples of simple vocal tics are making meaningless sounds, coughing, or mimicking animal noises. The symptoms vary greatly, so that each person affected shows their own appearance, which can also change over time.

The category of complex tics in the motor area includes imitating grimacing and imitating the actions of others ( echopraxia ). Self-injurious behavior , even with tic-like repetition, is attributed to other - possibly accompanying - disorders. Complex vocal tics are the repeating of words ( echolalia or palilalia ) or the hurling out of obscene and aggressive expressions known as coprolalia .

The symptoms can either appear permanently, several times a day (mostly in series) or only in stressful situations. The ability of many sufferers to suppress their tics over certain periods of time is also typical. It was found that they have an overall increased ability to control the initiation of movements compared to healthy people. This has been attributed to a training effect through the suppression of tics and corresponding adjustments in the brain. The exercise of suppression could therefore be a useful part of therapy.

Accompanying disturbances

The most common of the possible comorbidities is attention deficit / hyperactivity disorder (ADHD). Another common accompanying condition is obsessive-compulsive disorder . Other disorders occur, but it is often unclear whether these are not more of a part of the two main concomitant diseases mentioned above.

consequences

Social consequences

Those affected mainly suffer from the reaction of their environment to their symptoms. Precisely because people with Tourette's syndrome sometimes have little or no ability to influence their tic symptoms, the abnormalities associated with Tourette's syndrome are often interpreted as bad habits . As a result, parents of affected children often develop guilty feelings about their supposedly poor upbringing . The adolescents themselves encounter a lot of incomprehension and rejection in public and in school, which in turn can lead to an intensification of the abnormalities. Adults with Tourette's syndrome are also often discriminated against and often experience restrictions in their professional and private development. Outsiders often feel personally provoked by the involuntary tics. This is particularly noticeable in coprolalia and copropraxia and can lead to a worsening of such situations. Tourette patients are usually just as productive as their peers and, provided they do not have serious comorbidities, can fully participate in social life.

Special features in reaction and control

People with Tourette's syndrome have a slower response time to some tasks , which has been linked, among other things, to their exercise in motor control through the suppression of tics.

There are contradicting results on the question of motor dexterity, which was associated with possible unconsidered influences of accompanying disorders.

The neurologist and writer Oliver Sacks addresses the connection between Tourette and music in two chapters of his books. The syndrome is impulsive and productive. On the one hand it could lead to repetitive movements, on the other hand it could “take on an elaborate, phantasmagoric form”. People with phantasmagoric Tourette's syndrome could, if they succeeded in harnessing it, “display an exuberant and almost indomitable creativity ”. The American composer Tobias Picker , who has Tourette's syndrome, is free from tics while composing and making music. He himself believed that the tics had found their way into his creative imagination. The English pianist Nick van Bloss sees his Tourette syndrome as energy that he uses and channels while making music. According to Sacks, jazz and rock music are particularly attractive because of their heavy beats and the freedom to improvise .

Tourette and personality

In his various publications, Oliver Sacks addressed the relationship between Tourette's syndrome and the self of the person concerned. In the course of life, the syndrome develops an often complex interdependence with the personality. The relationship could be so destructive that some patients "in the face of the confusing chaos and the tremendous pressure of the impulses" could barely find their true identity. Other patients succeed in integrating the syndrome into their personality and "taking advantage of the frenzied pace of thoughts, associations and ideas that this syndrome brings with it." The syndrome can lead to "unusual and sometimes astonishing achievements" .

causes

Striatum in the human brain

Neurobiology

The basal ganglia , especially the striatum , have proven to be the central location of the disorders . The core areas of the caudate nucleus and the putamen in the latter are reduced in size in those affected. Imaging showed that their activity correlated with the frequency of tics . At the cellular level, investigations after death ( post mortem ) revealed a reduced number of parvalbumin- expressing and cholinergic interneurons .

Furthermore, there is extensive evidence that dopaminergic signal transmission in the striatum is disturbed.

genetics

Hereditary components for disease risk have been proven many times over, and they are stronger than in other neuropsychiatric diseases. Critical genetic deviations have not yet been found. It is assumed that a large number of discrepancies are involved and therefore so far (as of December 2015) no statements can be made about the heredity in specific individual cases.

immune system

A possible connection between deviations in the immune system and disorders in brain development, which could lead to an increased risk of developing Tourette's syndrome, have been investigated for many years. So far (as of December 2015), however, no reliable information is available.

course

Many of those affected by Tourette's syndrome in childhood experience symptoms subsiding in the course of or after puberty ; others also show the full picture of Tourette's syndrome as adults. Fluctuating courses with alternating degrees of severity are also known. However, Tourette's syndrome itself has no impact on life expectancy , even if it is chronic, and is not associated with mental deterioration .

diagnosis

The diagnosis of Tourette's syndrome is made on the basis of the symptoms observed and the previous course of the disease. Lighter courses in particular are often overlooked or incorrectly classified, so that it can take a few years for a correct diagnosis to be made. The German Society for Neurology recommends the following basic diagnostics:

  • Collection of medical history ( anamnesis )
  • Survey of the neurological status
  • A description of the type, frequency, intensity and distribution of involuntary movements and vocalizations as well as external influencing factors
  • Evaluation of the type and perception of perceptions (anticipation and suppressibility)
  • Survey of possible accompanying disorders such as ADHD , obsessive-compulsive symptoms, depression, anxiety, autoaggression

therapy

Primary tic disorders can still (as of 2015) neither be cured nor treated causally. Soothing treatments are available, but they do not cure the disease per se. The observable symptoms can mainly be reduced by treatment with psychotropic drugs from the group of neuroleptics , but most people with Tourette's syndrome are not so severely impaired that medication or other professional help is necessary. When assessing the effectiveness of a treatment strategy, strong fluctuations in the course of symptoms, such as are typical for Tourette's syndrome, also play a role. At the same time, studies on the treatment of tic disorders are still poor. Treatments are usually based on case reports .

Direct comparisons of the drugs are largely lacking; clear therapy recommendations cannot be derived from the data collected so far. In practice, treatment is therefore based on the experience of the treating specialist, the treatment costs and the approval status of the drug in question (often also off-label treatments). Drug treatment often leads to a moderate tic reduction (around 50%), but not to complete freedom from symptoms.

Psychoeducation and behavior therapy are used in addition. In the European guidelines for the diagnosis and treatment of Tourette's syndrome of 2011, clear criteria were specified for the first time as to when, how and with which medication treatment of the tics should be considered. In order to clarify individually tailored therapeutic measures, the specialist advice of doctors , preferably child and adolescent psychiatrists , psychiatrists or neurologists, should be sought.

Medication

If drug intervention is required due to the severity of the disease or accompanying disorders (e.g. obsessive-compulsive symptoms ) , various preparations are available. In Germany, tiapride or sulpiride are usually used. Pimozide and haloperidol are believed to have more side effects and are therefore only used as reserve drugs for severe tics. Risperidone is the best-studied drug, and by far the most widely used in Europe for treating tics, and is recommended as the drug of choice by the European Society for the Study of Tourette Syndrome (ESSTS). If tiapride, sulpiride and risperidone are out of the question, aripiprazole has recently been considered the closest alternative (as of January 2016). THC , one of the main active ingredients in cannabis , has been studied for possible use since 2001. However, a systematic review published by the Cochrane Collaboration in 2009 came to the conclusion that there was insufficient evidence to justify treatment with THC.

Psychotherapy and counseling

A special exercise method from the spectrum of behavior therapy , habit reversal training , has shown moderate (max. 30%) symptom relief for mild disease processes. Educational, special educational and curative education advice can also be helpful because of the concentration and attention disorders that occur frequently. Relaxation procedures are also available. The aim of these is to reduce stressful situations that lead to an intensification of the tics.

Positive results are also known from music therapy . Sometimes nervous impulses can be derived by playing an instrument. Fast instruments and instruments in which the player is active with hands and feet, e.g. B. the drums and the organ . The tendency to palipraxia also accommodates the constant repetition of phrases, bars and scales while practicing.

Surgical treatment

Deep brain stimulation is now successfully used in severe cases that cannot be treated otherwise, in Germany at appropriately specialized university clinics. However, the optimal target point for deep brain stimulation is still the subject of current research. In addition, it should be noted that the studies currently available on this topic only show very low patient numbers, so that a final assessment of the therapeutic benefit of deep brain stimulation is currently still difficult.

Aid organizations

Based on the US model, aid organizations were also formed in other countries, which generally set themselves the task of achieving more tolerance through information and education, but also to improve the training of specialist staff, so that both early medical detection and pedagogical handling of the symptoms are promoted become. In addition, help for self-help is propagated, and self-help groups offer the possibility of exchange among those affected.

In 1993, the Tourette-Gesellschaft Deutschland e. V. (TGD), followed in 2007 by the Tic & Tourette Syndrom e. V. (IVTS). In July 2010 the General Aid TIC & ADHS e. V. (AH-TA), which specializes in individual aid on site.

In Switzerland was 1,996 Tourette Society Switzerland (TGS) was founded.

Tourette Syndrome in Film, Literature, and the Public

  • The TV film Tics - My Annoying Companions (USA, 2008) tells the true story of the American Brad Cohen, who has suffered from Tourette's syndrome since childhood. After he was teased by his classmates, his environment and the doctors were initially at a loss and the teachers showed no understanding for his behavior, he later developed the desire to become a teacher himself in order to do better.
  • In the book Mr. Tourette and I , Pelle Sandstrak tells of his life with Tourette syndrome.
  • In the crime novel Wilsberg and the dead professor by Jürgen Kehrer , one of the characters describes himself as a "Touretter". He is described in detail with his linguistic tics. In the novel Coma by John Niven a protagonist after an accident to "Tour Etter" is.
  • In the film Vincent will Meer , Florian David Fitz plays a young man suffering from Tourette's syndrome. Another film that addresses this syndrome is the comedy Ein Tick anders with Jasna Fritzi Bauer .
  • In the series South Park Eric claims subsequently Petit Tourette to be sick syndrome Tourette on to all the people with impunity insult around him to. The character Marty Fisher in the series Shameless also suffers from Tourette's syndrome , with a very pronounced coprolalia .
  • In the feature film The Square (Sweden et al., 2017) there is a scene in which, during a panel discussion about an art exhibition, the obscene verbal failures of a person allegedly affected by Tourette's syndrome affected the "tolerance" and "mindfulness" of the other audience, especially a female Challenge the discussant.
  • Olaf Blumberg: I rarely say fuck. My life with Tourette . Ullstein 2013, ISBN 978-3-550-08012-8 (autobiographical).
  • Jonathan Lethem : Motherless Brooklyn . Goldmann, Munich 2004, ISBN 3-442-54187-5 (novel). In 2019, the novel was filmed as Motherless Brooklyn, with Edward Norton as director and lead actor. Actor Bruce Willis is involved in the film .
  • Timothy Matthew "Tim" Howard , the US soccer goalkeeper and international player, has made his Tourette disease public in order to raise public awareness of Tourette's syndrome.
  • Anne Heche plays in some episodes of the US TV series " Ally McBeal " from season 4 / episode 9 the role of Melanie West, a woman who suffers from Tourette's syndrome and who runs over her during a seizure after an argument with her boyfriend. She is on trial for this “murder”.
  • Mirjam Mous: Virus - Those who give up have lost. A youth novel whose protagonist suffers from Tourette's syndrome.
  • The YouTube channel Gewitter im Kopf - Leben mit Tourette , which is run by a Tourette sufferer, has gained over 1.6 million subscribers since February 2019. The channel deals with Tourette's syndrome in a humorous way and is intended to educate viewers and help to break down prejudices, fears and misunderstandings.
  • 10 questions to a Tourette patient from Galileo / ProSieben
  • Radio play Chinchilla Arschloch, waswas by Helgard Haug and Thilo Guschas (WDR 2018, German Radio Play Award of ARD 2019)
  • The American grunge rock band Nirvana released a song called Tourette's on their album In Utero in 1993 .

See also

literature

history

  • Gilles de la Tourette : The hysteria according to the teachings of the Salpêtrière . A study by Gilles de la Tourette, after whom Tourette syndrome is named. Reprint of the original from 1894. Outlook, Bremen 2012, ISBN 3-86403-451-5 .

Introductions

  • Kirsten R. Müller-Vahl: Tourette syndrome and other tic diseases in childhood and adulthood . 2nd updated and expanded edition, Medizinisch Wissenschaftliche Verlagsgesellschaft, Berlin 2014, ISBN 978-3-95466-099-5 .
  • James F. Leckman, Donald J. Cohen: Tourette's Syndrome Tics, Obsessions, Compulsions: Developmental Psychopathology and Clinical Care . John Wiley & Sons, New York 2002, ISBN 0-471-11375-1 .

Advisory literature

  • Uttom Chowdhury, Isobel Heyman: Tics and Tourette Syndrome: A Handbook for Parents and Professionals . Jessica Kingsley Publishers, New York 2004, ISBN 1-84310-203-X .
  • Manfred Döpfner, Veit Roessner, Katrin Woitecki, Aribert Rothenberger: Tic disorders: counselor for child and adolescent psychotherapy . Hogrefe, Göttingen 2010, ISBN 3-8409-1728-X .
  • Susann Sulzbach: The Tourette syndrome in the context of language therapy: An overview for teachers, educators and parents . Disserta, Hamburg 2015, ISBN 3-95425-892-7 .

Web links

Wiktionary: Tourette's syndrome  - explanations of meanings, word origins, synonyms, translations

Guidelines

Advice

Individual evidence

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  2. G. Gilles de la Tourette: Étude sur une affection nerveuse characterisée par de l'incoordination motrice accompagnée d'écholalie et de coprolalie (jumping, latah, myriachit). In: Arch Neurol (Paris) . tape 9 , 1885, p. 19-42 and 158-200 .
  3. G. Gilles de la Tourette: Étude sur une affection nerveuse characterisée par de l'incoordination motrice accompagnée d'écholalie et de coprolalie. (Jumping, Latah, Myriachit, maladie des tics convulsifs, maladie de Gilles de la Tourette). Paris, Delahaye et Lecrosnier 1885: Étude sur une affection nerveuse characterisée par de l'incoordination motrice accompagnée d'écholalie et de coprolalie (Jumping, Latah, Myriachit, maladie des tics convulsifs, maladie de Gilles de la Tourette). Delahaye et Lecrosnier, Paris 1885.
  4. ^ Itard JMG .: Mémoire sur quelques fonctions involuntaires des appareils de la locomotion, de la préhension et de la voix. In: Arch Gén Méd (Paris) . tape 8 , 1825, p. 385-407 .
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  22. a b Oliver Sacks: The life of a surgeon . In: An Anthropologist on Mars p. 119.
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  51. Radio play: No intention, just Tourette. Chinchilla asshole what what. deutschlandfunkkultur.de, December 12, 2019