title: Deep brain stimulation in childhood: an effective treatment for early onset idiopathic generalised dystonia.
abstract: BACKGROUND
  Early onset idiopathic generalised dystonia is a progressive and profoundly disabling condition. Medical treatment may ameliorate symptoms. However, many children have profound, intractable disability including the loss of ambulation and speech, and difficulties with feeding. Following the failure of medical management, deep brain stimulation (DBS) of the globus pallidus internus (GPi) has emerged as an alternative treatment for the disorder.
  
  
  METHODS
  We describe four children who presented with dystonia.
  
  
  RESULTS
  Following the failure of a range of medical therapies, DBS systems were implanted in the GPi in an attempt to ameliorate the children's disabilities. All children found dystonic movements to be less disabling following surgery. Compared with preoperative Burke, Fahn and Marsden Dystonia Rating Scale scores, postoperative scores at 6 months were improved.
  
  
  CONCLUSIONS
  DBS is effective in improving symptoms and function in children with idiopathic dystonia refractory to medical treatment. Whilst surgery is complex and can be associated with intraoperative and postoperative complications, this intervention should be considered following the failure of medical therapy.
authors: Department of Paediatric Neurology, Children's Hospital, Oxford, UK. jeremyparr@doctors.org.uk
authors: Jeremy R
authors: JR
authors: Parr
authors: Alex L
authors: AL
authors: Green
authors: Carole
authors: C
authors: Joint
authors: Morag
authors: M
authors: Andrew
authors: Ralph P
authors: RP
authors: Gregory
authors: Richard B
authors: RB
authors: Scott
authors: Michael A
authors: MA
authors: McShane
authors: Tipu Z
authors: TZ
authors: Aziz
mesh_terms: D000293:Adolescent; D002648:Child; D046690:Deep Brain Stimulation / Q000379:methods*; D020821:Dystonic Disorders / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D005917:Globus Pallidus / Q000601:surgery; D006801:Humans; D008297:Male; D016896:Treatment Outcome