title: German registry of paediatric deep brain stimulation in patients with childhood-onset dystonia (GEPESTIM).
abstract: 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.
  
  
  OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurology, University Hospital of Cologne, Germany; Department of Paediatrics, University Hospital of Cologne, Germany. Electronic address: anne.koy@uk-koeln.de.
authors.forename: A
authors.initials: A
authors.lastname: Koy
authors.affiliation: Department of Neurology, University Hospital of Cologne, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Weinsheimer
authors.affiliation: Department of Neurology, University Hospital of Cologne, Germany.
authors.forename: K A M
authors.initials: KA
authors.lastname: Pauls
authors.affiliation: Department of Neurology, Charité University Medicine Berlin, Germany.
authors.forename: A A
authors.initials: AA
authors.lastname: Kühn
authors.affiliation: Department of Neurology, Charité University Medicine Berlin, Germany.
authors.forename: P
authors.initials: P
authors.lastname: Krause
authors.affiliation: Department of Neurology, Charité University Medicine Berlin, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Huebl
authors.affiliation: Department of Neurosurgery, Charité University Medicine Berlin, Germany.
authors.forename: G-H
authors.initials: GH
authors.lastname: Schneider
authors.affiliation: Department of Neurology, University Hospital of Schleswig-Holstein, Campus Kiel, Germany.
authors.forename: G
authors.initials: G
authors.lastname: Deuschl
authors.affiliation: Department of Neurology, University Hospital of Schleswig-Holstein, Campus Kiel, Germany.
authors.forename: R
authors.initials: R
authors.lastname: Erasmi
authors.affiliation: Department of Neurosurgery, University Hospital of Schleswig-Holstein, Campus Kiel, Germany.
authors.forename: D
authors.initials: D
authors.lastname: Falk
authors.affiliation: Department of Neurosurgery, Medical School, MHH, Hannover, Germany.
authors.forename: J K
authors.initials: JK
authors.lastname: Krauss
authors.affiliation: Department of Neurosurgery, Medical School, MHH, Hannover, Germany.
authors.forename: G
authors.initials: G
authors.lastname: Lütjens
authors.affiliation: Department of Neurology, University Hospital of Düsseldorf, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Schnitzler
authors.affiliation: Department of Neurology, University Hospital of Düsseldorf, Germany.
authors.forename: L
authors.initials: L
authors.lastname: Wojtecki
authors.affiliation: Department of Stereotactic and Functional Neurosurgery, University Hospital of Düsseldorf, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Vesper
authors.affiliation: Department of Paediatrics, Freiburg University Medical Centre, Germany.
authors.forename: R
authors.initials: R
authors.lastname: Korinthenberg
authors.affiliation: Department Stereotactic and Functional Neurosurgery, Freiburg University Medical Centre, Germany.
authors.forename: V A
authors.initials: VA
authors.lastname: Coenen
authors.affiliation: Department of Stereotactic and Functional Neurosurgery, University Hospital of Cologne, Germany.
authors.forename: V
authors.initials: V
authors.lastname: Visser-Vandewalle
authors.affiliation: Institute of Medical Statistics, Informatics and Epidemiology, University of Cologne, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Hellmich
authors.affiliation: Department of Neurology, University Hospital of Cologne, Germany. Electronic address: lars.timmermann@uk-koeln.de.
authors.forename: L
authors.initials: L
authors.lastname: Timmermann
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D046690:Deep Brain Stimulation; D020821:Dystonic Disorders / Q000209:etiology / Q000503:physiopathology / Q000534:rehabilitation*; D005260:Female; D005858:Germany; D005917:Globus Pallidus / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D008297:Male; D015337:Multicenter Studies as Topic; D009460:Neurologic Examination; D012042:Registries; D012720:Severity of Illness Index; D016896:Treatment Outcome
keywords: Deep brain stimulation; Dystonia; Paediatric; Registry