Neurobiologische Grundlagen, Klinik und Therapie des Tourette-Syndroms

Zusammenfassung

Das Tourette-Syndrom ist gekennzeichnet durch die Leitsymptome motorische und vokale Tics. Ein typischer klinischer Verlauf besteht aus Erstmanifestation im Kindesalter und Rückbildung der Symptomatik bei nahezu zwei Dritteln der Betroffenen bis zum jungen Erwachsenenalter. Angesichts dieser Entwicklung rücken pathophysiologisch die Entwicklung und Reifung von neuronalen Netzwerken in den Vordergrund. Als neuronales pathophysiologisches Korrelat steht der kortiko-striato-thalamo-kortikale Kreislauf seit Langem im Fokus der Tourette-Forschung. Den Basalganglien wird in der Entstehung von Tics eine wichtige Rolle zugesprochen. Die motorischen Anteile der Basalganglien kontrollieren die Geschwindigkeit und Richtung einer willkürlichen Bewegung. Diese zwei Kernelemente sind bei Tics verändert. Obwohl Tics die Kardinalsymptome des Tourette-Syndroms darstellen, spielen im Alltag der Patienten eine emotionale Dysregulation und ein hohes Maß an Impulsivität eine große Rolle und tragen sehr zur Beeinträchtigung durch die Störung bei. Diese weitergehenden Verhaltensauffälligkeiten sind wiederum eng mit der Frequenz und Intensität von Tics verbunden. Hänseleien z. B. an der Bushaltestelle lassen die Ticfrequenz deutlich ansteigen, Tätigkeiten, die Konzentration erfordern, wie Lesen, lassen die Ticfrequenz sinken. Die Ergebnisse struktureller und funktioneller bildgebender Verfahren stützen die Hypothese einer veränderten Signaltransmission in den Basalganglien und einer Affektion des limbischen Systems. Sie zeigen jedoch auch eine komplexe Interaktion zwischen prämotorischem Kortex, supplementär motorischem Kortex, anteriorem Cingulum, präfrontalem Kortex und Basalganglien auf.

Abstract

Tourette’s syndrome (TS) is characterised by motor and vocal tics. The clinical manifestation during childhood and the frequent remission during early adulthood point to a dysregulation in the maturation of neuronal pathways. As a neurobiological correlate, the cortico-striato-thalamo-cortical circuit is the main focus of research. Two main features of a movement, the speed and direction of a movement, are controlled by the basal ganglia. Both features are dysregulated in TS. Besides the motor symptoms, patients with TS suffer from comorbidities such as attention deficit hyperactivity syndrome, depression and obsessive compulsive disorders. Tics are modified in frequency and intensity by actions that require a high level of concentration such as reading or by distress, e. g., teasing by peers. The results of structural and functional imaging data support the hypothesis of altered signal transmission in the basal ganglia and a dysfunction in the limbic system. They also point to a complex interaction between cortical motor areas, the anterior cingulum, prefrontal regions and the basal ganglia.

Schlüsselwörter

Tics - Tourette - MR-Bildgebung

Key words

tics - Tourette’s syndrome - imaging

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