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Deep brain stimulation in childhood: An effective treatment for early onset generalised idiopathic dystonia
  1. Jeremy R Parr (jeremyparr{at}doctors.org.uk)
  1. Department of Paediatric Neurology, The John Radcliffe Hospital, Oxford, United Kingdom
    1. Alex L Green
    1. Department of Neurosurgery, Radcliffe Infirmary, Oxford, United Kingdom
      1. Carole Joint
      1. Department of Neurosurgery, Radcliffe Infirmary, Oxford, United Kingdom
        1. Morag Andrew
        1. Department of Paediatric Neurology, The John Radcliffe Hospital, Oxford, United Kingdom
          1. Ralph P Gregory
          1. Department of Neurology, Radcliffe Infirmary, Oxford, United Kingdom
            1. Richard B Scott
            1. Department of Clinical Psychology, Radcliffe Infirmary, Oxford, United Kingdom
              1. Michael A McShane
              1. Department of Paediatric Neurology, The John Radcliffe Hospital, Oxford, United Kingdom
                1. Tipu Z Aziz
                1. Department of Neurosurgery, Radcliffe Infirmary, Oxford, United Kingdom

                  Abstract

                  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, 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. We describe 4 children who presented with dystonia. Following the failure of a range of medical therapies, DBS systems were implanted in the GPi in an attempt to ameliorate their condition. 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 significantly improved. 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 intra operative and postoperative complications, this intervention should be considered following the failure of medical therapy.

                  • Deep Brain Stimulation
                  • Globus Pallidus Internus (GPi)
                  • idiopathic generalised dystonia
                  • neurodisability
                  • torsion dystonia

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