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Tourette’s syndrome: a cross sectional study to examine the PANDAS hypothesis
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  1. A J Church1,
  2. R C Dale3,
  3. A J Lees4,
  4. G Giovannoni1,
  5. M M Robertson2
  1. 1Neuroinflammation Department, Institute of Neurology, Queen Square, London, UK
  2. 2Division of Neuropsychiatry, Institute of Neurology
  3. 3Neurosciences Unit, Institute of Child Health, London, UK
  4. 4Reta Lila Weston Institute of Neurological Studies, Royal Free and University College Hospital Medical School, London, UK
  1. Correspondence to:
 Mr Andrew Church, Neuroimmunology Unit, Room 917, Institute of Neurology, Queen Square, London WC1N 3BG, UK; 
 a.church{at}ion.ucl.ac.uk

Abstract

Background: The classical neurological disorder after group A β haemolytic streptococcal infection is Sydenham’s chorea. Recently a tic disorder occurring after group A streptococcal infection has been described and termed PANDAS (paediatric autoimmune neuropsychiatric disorders associated with streptococcal infection). It is proposed that antibodies induced after group A streptococcal infection react with basal ganglia neurones in Sydenham’s chorea and PANDAS. Anti-basal ganglia antibodies (ABGA) are present in most cases of acute Sydenham’s chorea, but rarely in controls.

Objective: To investigate the hypothesis that Tourette’s syndrome may be associated with group A streptococcal infection and ABGA.

Methods: 100 patients with Tourette’s syndrome (DSM-IV-TR) were enrolled in a cross sectional study. Children with neurological disease (n = 50) and recent uncomplicated streptococcal infection (n = 40), adults with neurological disease (n = 50), and healthy adults (n = 50) were studied as controls. Recent group A streptococcal infection was defined using antistreptolysin O titre (ASOT). ABGA were detected using western immunoblotting and indirect immunofluorescence.

Results: ASOT was raised in 64% of children with Tourette’s syndrome compared with 15% of paediatric neurological disease controls (p < 0.0001), and in 68% of adults with Tourette’s syndrome compared with 12% of adult neurological controls and 8% of adult healthy controls (p < 0.05). Western immunoblotting showed positive binding in 20% of children and 27% of adults with Tourette’s syndrome, compared with 2–4% of control groups (p < 0.05). The most common basal ganglia binding was to a 60 kDa antigen, similar to the proposed antigen in Sydenham’s chorea. Indirect immunofluorescence revealed autoantibody binding to basal ganglia neurones. Serological evidence of recent group A streptococcal infection, assessed by a raised ASOT, was detected in 91% (21/23) of Tourette’s syndrome patients with positive ABGA compared with 57% (44/77) with negative ABGA (p < 0.01).

Conclusions: The results support a role of group A streptococcal infection and basal ganglia autoimmunity in a subgroup of patients with Tourette’s syndrome and suggest a pathogenic similarity between Sydenham’s chorea and some patients with Tourette’s syndrome.

  • Tourette syndrome
  • streptococcal infection
  • anti-basal ganglia antibodies
  • ABGA, anti-basal ganglia antibodies
  • ADHD, attention deficit hyperactivity disorder
  • ASOT, antistreptolysin O titre
  • DSM, Diagnostic and Statistical Manual of Mental Disorders
  • ICD, International Classification of Diseases
  • PANDAS, paediatric autoimmune neuropsychiatric disorders associated with streptococcal infection
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Footnotes

  • Competing interests: none declared

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