German registry of paediatric deep brain stimulation in patients with childhood-onset dystonia (GEPESTIM)
A Koy1, M Weinsheimer2, K A M Pauls2
1Department of Neurology, University Hospital of Cologne, Germany; Department of Paediatrics, University Hospital of Cologne, Germany.
Insights
Deep brain stimulation (DBS) shows positive effects for paediatric dystonia, particularly inherited and acquired forms. Long-term outcomes require further study, with hardware issues like battery replacement being notable.
Area of Science:
- Neurology
- Neurosurgery
- Pediatric Medicine
Background:
- Limited data exists on pediatric deep brain stimulation (DBS), especially long-term outcomes.
- Small single-center studies and lack of multi-center trials contribute to data scarcity.
Purpose of the Study:
- To systematically evaluate the clinical outcomes of pediatric patients undergoing DBS.
- To establish a German registry (GEPESTIM) for pediatric DBS data collection.
Main Methods:
- Collected data from 44 pediatric dystonia patients (under 18) across 6 German centers.
- Classified patients into inherited, acquired, and idiopathic dystonia groups.
- Analyzed pre- and postoperative Burke-Fahn-Marsden Dystonia Rating Scale scores.
Main Results:
- Significant improvement in dystonia scores post-DBS (average absolute improvement 15.5 ± 18.0, p < 0.001).
- Infection was the primary reason for hardware removal (n=6).
- 15 patients required 20 IPG replacements due to battery expiry (3.7 ± 1.8 years post-implantation).
Conclusions:
- Pediatric DBS demonstrates positive effects on inherited and acquired dystonia.
- Adverse events, including frequent IPG replacements, are prominent in pediatric dystonia patients.
- The GEPESTIM registry facilitates collaborative research and future standardized management algorithms for pediatric DBS.
Background:
Data on paediatric deep brain stimulation (DBS) is limited, especially for long-term outcomes, because of small numbers in single center series and lack of systematic multi-center trials.
Objectives:
We seek to systematically evaluate the clinical outcome of paediatric patients undergoing DBS.
Methods:
A German registry on paediatric DBS (GEPESTIM) was created to collect data of patients with dystonia undergoing DBS up to the age of 18 years. Patients were divided into three groups according to etiology (group 1 inherited, group 2 acquired, and group 3 idiopathic dystonia).
Results:
Data of 44 patients with a mean age of 12.8 years at time of operation provided by 6 German centers could be documented in the registry so far (group 1 n = 18, group 2 n = 16, group 3 n = 10). Average absolute improvement after implantation was 15.5 ± 18.0 for 27 patients with pre- and postoperative Burke-Fahn-Marsden Dystonia Rating scale movement scores available (p < 0.001) (group 1: 19.6 ± 19.7, n = 12; group 2: 7.0 ± 8.9, n = 8; group 3: 19.2 ± 20.7, n = 7). Infection was the main reason for hardware removal (n = 6). 20 IPG replacements due to battery expiry were necessary in 15 patients at 3.7 ± 1.8 years after last implantation.
Discussion:
Pre- and postoperative data on paediatric DBS are very heterogeneous and incomplete but corroborate the positive effects of DBS on inherited and acquired dystonia. Adverse events including relatively frequent IPG replacements due to battery expiry seem to be a prominent feature of children with dystonia undergoing DBS. The registry enables collaborative research on DBS treatment in the paediatric population and to create standardized management algorithms in the future.
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