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Arch Dis Child 2008;93:239-240 doi:10.1136/adc.2007.118968
  • Case report

Pallidal stimulation for pantothenate kinase-associated neurodegeneration dystonia

  1. C Isaac1,2,
  2. I Wright2,3,
  3. D Bhattacharyya4,
  4. P Baxter3,
  5. J Rowe4
  1. 1
    Clinical Neuropsychology Services, Royal Hallamshire Hospital, Sheffield, UK
  2. 2
    Clinical Psychology Unit, Department of Psychology, University of Sheffield, Sheffield, UK
  3. 3
    Ryegate Children’s Centre, Sheffield Children’s NHS Trust, Sheffield, UK
  4. 4
    Department of Neurosurgery, Royal Hallamshire Hospital, Sheffield, UK
  1. Jeremy Rowe, Department of Neurosurgery, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF, UK; Jeremy.Rowe{at}sth.nhs.uk
  • Accepted 29 June 2007

Abstract

Pantothenate kinase-associated neurodegeneration is associated with generalised dystonia and cognitive deterioration. Limited evidence suggests that pallidal deep brain stimulation improves physical functioning. This is a report of the assessment and treatment of a severely affected patient in whom pallidal deep brain stimulation improved both physical and psychosocial functioning. Implications for treatment are briefly discussed.

Footnotes

  • Competing interests: None.

  • Patient consent: Consent has been obtained for publication of fig 1.

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