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Late recurrences of Sydenham’s chorea are not associated with anti-basal ganglia antibodies
  1. N A Harrison2,
  2. A Church1,
  3. A Nisbet4,
  4. P Rudge3,
  5. G Giovannoni1
  1. 1Neuroimmunology Unit, Neuroinflammation, Institute of Neurology, Queen Square, London, UK
  2. 2Psychiatry, Institute of Psychiatry, De Crespigny Park, London, UK
  3. 3Neurology, National Hospital of Neurology and Neurosurgery, Queen Square, London, UK
  4. 4Neurology, Royal Sussex County Hospital, Brighton, UK
  1. Correspondence to:
 Dr G Giovannoni
 Neuroimmunology Unit, Neuroinflammation, Institute of Neurology, Queen Square, London, WC1N 3BG, UK; G.Giovannoniion.ucl.ac.uk

Abstract

Anti-basal ganglia antibodies (ABGA) have been associated with 100% of acute cases and 69% of persistent cases of Sydenham’s chorea. We describe two cases of late recurrences of Sydenham’s chorea with absence of ABGA. Both patients had several childhood episodes of Sydenham’s chorea. MRI imaging of the basal ganglia and exhaustive investigations for other causes of chorea were normal or negative. The absence of ABGA may be evidence against an autoimmune pathology in late and some persistent recurrences. We suggest the likely pathophysiology to be dopamine hypersensitivity of chronically damaged basal ganglia neurones possibly following induction of an autoimmune antibody response in childhood.

  • ABGA, anti-basal ganglia antibodies
  • SC, Sydenham’s chorea
  • anti-basal ganglia antibodies
  • St Vitus dance
  • Sydenham’s chorea

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Footnotes

  • Competing interests: none declared