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Deep brain stimulation in childhood: an effective treatment for early onset idiopathic generalised dystonia
  1. Jeremy R Parr1,
  2. Alex L Green2,
  3. Carole Joint2,
  4. Morag Andrew,
  5. Ralph P Gregory2,
  6. Richard B Scott3,
  7. Michael A McShane1,
  8. Tipu Z Aziz2
  1. 1Department of Paediatric Neurology, Children’s Hospital, Oxford, UK
  2. 2Department of Neurosurgery, John Radcliffe Hospital, Oxford, UK
  3. 3Russell Cairns Unit, John Radcliffe Hospital, Oxford, UK
  1. Correspondence to:
    Dr Jeremy Parr
    University of Oxford Department of Paediatrics, Children’s Hospital, Oxford OX3 9DU, UK; jeremyparr{at}doctors.org.uk

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.

  • BFMDRS, Burke, Fahn and Marsden Dystonia Rating Scale
  • DBS, deep brain stimulation
  • DRD, dopa responsive dystonia
  • IGD, idiopathic generalised dystonia
  • GPi, globus pallidus internus
  • IPG, implantable pulse generator
  • generalised dystonia
  • torsion dystonia
  • deep brain stimulation
  • globus pallidus internus (GPi)
  • neurodisability

https://doi.org/10.1136/adc.2006.095380

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    Footnotes

    • ↵i BFMDRS scores26 comprise a severity score (range 0–120) and a functional disability score (range 0–30). Whilst BFMDRS validation studies have not been completed in children, the scale is the most frequently used measure of the efficacy of DBS in childhood.27282930313233 The severity score is calculated by combining the degree of provocation required to cause dystonia (ranging from no dystonia at rest, to dystonia present at rest) and the severity in various muscle groups (eyes, mouth, speech and swallowing, neck, arm, trunk and leg). The functional disability score represents the degree of disability caused to speech, eating/swallowing, feeding, walking, handwriting, hygiene and dressing.

    • Published Online First 25 April 2007

    • Competing interests: None.

    • Informed consent was obtained for the video available online at http://www.archdischild.com/supplemental.

    Linked Articles

    • Précis
      A brief digest of the August issue
      BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health
      Archives of Disease in Childhood 2007; 92 e8-e8 Published Online First: 21 Jul 2007.