Tiefe Hirnstimulation bei sekundären Dystonien im Kindesalter

Zusammenfassung

Sekundäre Dystonien im Kindesalter umfassen eine heterogene Krankheitsgruppe mit unterschiedlichen Ätiologien. Die dyskinetische Ze­rebralparese ist die häufigste Ursache. Pharmakotherapie ist oftmals ohne Effekt oder die Nebenwirkungen werden nicht toleriert. In den letzten Jahren haben einige Patienten mit sekundärer Dystonie eine Tiefe Hirnstimulation (THS) erhalten. Die Ergebnisse sind sehr unterschiedlich und reichen von sehr gutem Ansprechen bis hin zu keinem Effekt. Patienten, die sich anhand klinischer Dystonie-Skalen kaum verbessern, berichten oftmals von einer Verbesserung der Lebensqualität. Es gibt wenige Daten zur Anwendung im Kindesalter. Die Erfahrungen zur THS bei Patienten mir primärer oder idiopathischer Dystonie weisen darauf hin, dass die THS zu einem frühen Zeitpunkt des Krankheitsverlaufs oftmals mit einem größeren Effekt assoziiert ist. Dennoch ist die Indikation zur THS im Kindesalter sorgfältig abzuwägen. Für die Zukunft sind größere prospektive Studien mit jungen, gut charakterisierten, homogenen Patientenkohorten erforderlich, die den Effekt der THS auf Bereiche wie Motorik und Lebensqualität systematisch erfassen, um die Bedeutung der THS als Therapieoption für Patienten mit sekundärer Dystonie weiter zu evaluieren.

Abstract

Secondary dystonia encompasses a heterogeneous group of diseases with different aetiologies. Cerebral palsy is the most common cause. Pharmacological treatment is often unsatisfactory or side effects are not well tolerated. During the last decade some patients with secondary dystonia were treated with deep brain stimulation (DBS) – with varying results ranging from a good response to no effect. Patients without measurable response in clinical rating scales for dystonia sometimes report improvements in quality of life. The data on paediatric DBS are very limited. According to the experiences with DBS in patients with primary or idiopathic dystonia, DBS at an early stage of disease progression is associated with a greater improvement. Still, the indication for DBS in children has to be considered carefully. In the future, larger prospective studies with young, well-characterised and aetiologically homogeneous cohorts of patients with secondary dystonia are necessary to systematically investigate the effect of DBS on motor function and quality of life to further evaluate the role of DBS in secondary dystonia.

Schlüsselwörter

kindliche Bewegungsstörungen - sekundäre Dystonie - Tiefe Hirnstimulation

Key words

paediatric movement disorders - secondary dystonia - deep brain stimulation

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