Details

Autor(en) / Beteiligte

• Koy, A., MD
• Weinsheimer, M
• Pauls, K.A.M., MD, PhD
• Kühn, A.A., MD
• Krause, P., MD
• Huebl, J., MD
• Schneider, G.-H., MD
• Deuschl, G., MD, PhD
• Erasmi, R., MD
• Falk, D., MD
• Krauss, J.K., MD
• Lütjens, G., MD
• Schnitzler, A., MD
• Wojtecki, L., MD
• Vesper, J., MD, PhD
• Korinthenberg, R., MD
• Coenen, V.A., MD
• Visser-Vandewalle, V., MD, PhD
• Hellmich, M., PhD
• Timmermann, L., MD

Titel

German registry of paediatric deep brain stimulation in patients with childhood-onset dystonia (GEPESTIM)

Ist Teil von

• European journal of paediatric neurology, 2017-01, Vol.21 (1), p.136-146

Ort / Verlag

England: Elsevier Ltd

Erscheinungsjahr

2017

Quelle

MEDLINE

Beschreibungen/Notizen

• Abstract Background Data on paediatric 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 and 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=23, group 2 n=16, group 3 n=5). 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: 21.2±19.9, n=15; group 2: 7.0±8.9, n=8; group 3: 13.0±19.4, n=4). Infection was the main reason for hardware removal (n= 6). 20 IPG replacements due to battery expiry were necessary in 15 patients 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 effect of DBS of the Globus pallidus internus on inherited and acquired dystonia. Side effects seem to be a prominent feature of children with dystonia undergoing DBS as are relatively frequent replacements of IPGs due to battery expiry. The registry enables collaborative research on DBS treatment in the paediatric population and to create standardized treatment algorithms in the future.