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A dystonic syndrome associated with anti-basal ganglia antibodies
  1. M J Edwards1,
  2. R C Dale2,3,
  3. A J Church2,
  4. G Giovannoni2,
  5. K P Bhatia1
  1. 1Sobell Department of Movement Neuroscience and Movement Disorders, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK
  2. 2Neuroimmunology Unit, Department of Neuroinflammation, Institute of Neurology, University College London
  3. 3Neurosciences Unit, Institute of Child Health, Guilford Street, London WC1N 3JJ, UK
  1. Correspondence to:
 Dr K Bhatia
 Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London WC1N 3BG, UK; k.bhatiaion.ucl.ac.uk

Abstract

Anti-basal ganglia antibodies (ABGA) have been associated with movement disorders (usually tics and chorea) and psychiatric disturbance in children. This report describes five adult and adolescent patients (one male, four females; mean age of onset, 16 years (range, 13–35)) who presented subacutely with a clinical syndrome dominated by dystonia and had ABGA binding to antigens of similar molecular weights to those seen in Sydenham’s chorea. Three patients had a clear history of respiratory infection before the onset of their symptoms. Three patients received immunosuppressive treatment, with three showing a notable reduction in symptoms. It is hypothesised that dystonia in adults or adolescents may be part of the clinical spectrum of the post-infectious syndrome associated with ABGA.

  • anti-basal ganglia antibodies
  • dystonias
  • paediatric autoimmune neuropsychiatric disorders associated with streptococcal infection
  • Sydenham’s chorea
  • ABGA, anti-basal ganglia antibodies
  • ELISA, enzyme linked immunosorbent assay
  • ivIg, intravenous immunoglobulin
  • MRI, magnetic resonance imaging
  • PANDAS, paediatric autoimmune neuropsychiatric disorder associated with streptococcal infection
  • SC, Sydenham’s chorea
  • TS, Tourette’s syndrome

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Footnotes

  • Competing interests: none declared