Deep Brain Stimulation in Secondary Dystonia in Childhood

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.