Sydenham’s Chorea, PANDAS, and Other Poststreptococcal Neurological Disorders

  • Roser Pons
Part of the Current Clinical Neurology book series (CCNEU)


A 12-year-old boy developed hyperthyroidism secondary to Grave’s disease, which was successfully treated with I-131 treatment. Several months later, pharyngeal infection with group A β-hemolytic Streptococcus was documented by throat culture and subsequent rise in antistreptolysin O titer. He was treated with oral antibiotics, and 2 weeks later developed insidious, progressive chorea, incoordination with right hemibody, and imbalance. Examination revealed moderate chorea affecting the eyes, arms, and legs, incoordination of fine hand movements, motor impersistence on hand grip and tongue protrusion, and near inability to walk. He was treated with valproic acid, and his symptoms resolved within 3 weeks.


Attention Deficit Hyperactivity Disorder Valproic Acid Rheumatic Fever Streptococcal Infection Streptococcal Pharyngitis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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  1. 1.
    Jummani R, Okun M. Sydenham chorea. Neurology 2001;58:311–313.CrossRefGoogle Scholar
  2. 2.
    Shiffman RN. Guideline maintenance and revision. 50 years of the Jones criteria for diagnosis of rheumatic fever. Arch Pediatr Adolesc Med 1995;149:727–732.PubMedGoogle Scholar
  3. 3.
    Swedo SE, Rapoport JL, Cheslow DL, et al. High prevalence of obsessive-compulsive symptoms in patients with Sydenham’s chorea. Am J Psychiatry 1989;146:246–249.PubMedGoogle Scholar
  4. 4.
    Swedo SE. Sydenham’s chorea. A model for childhood autoimmune neuropsychiatric disorders. JAMA 1994;272:1788–1791.PubMedCrossRefGoogle Scholar
  5. 5.
    Swedo SE, Leonard HL, Garvey M, et al. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases. Am J Psychiatry 1998;155:264–271.PubMedGoogle Scholar
  6. 6.
    Dale RC, Church AJ, Cardoso F, et al. Poststreptococcal acute disseminated encephalomyelitis with basal ganglia involvement and auto-reactive antibasal ganglia antibodies. Ann Neurol 2001;50:588–595.PubMedCrossRefGoogle Scholar
  7. 7.
    DiFazio MP, Morales J, Davis R. Acute myoclonus secondary to group A beta-hemolytic streptococcus infection: a PANDAS variant. J Child Neurol 1998;13:516–518.PubMedGoogle Scholar
  8. 8.
    Dale RC, Church AJ, Benton S, et al. Post-streptococcal autoimmune dystonia with isolated bilateral striatal necrosis. Dev Med Child Neurol 2002;44:485–489.PubMedCrossRefGoogle Scholar
  9. 9.
    Dale RC, Church AJ, Surtees RA, Thompson EJ, Giovannoni G, Neville BGR. Post-streptococcal autoimmune neuropsychiatric disease presenting as paroxysmal dystonic choreoathetosis. Mov Disord 2002;17:817–820.PubMedCrossRefGoogle Scholar
  10. 10.
    Dale RC. Autoimmunity and the basal ganglia: new insights into old diseases. QJM 2003;96:183–191.PubMedCrossRefGoogle Scholar
  11. 11.
    Cardoso F, Eduardo C, Silva AP, Mota CC. Chorea in fifty consecutive patients with rheumatic fever. Mov Disord 1997;12:701–703.PubMedCrossRefGoogle Scholar
  12. 12.
    Carapetis JR, Currie BJ. Rheumatic fever in a high incidence population: the importance of monoarthritis and low grade fever. Arch Dis Child 2001;85:223–227.PubMedCrossRefGoogle Scholar
  13. 13.
    Terreri MT, Roja SC, Len CA, Faustino PC, Roberto AM, Hilario MO. Sydenham’s chorea—clinical and evolutive characteristics. Sao Paulo Med J 2002;120:16–19.PubMedCrossRefGoogle Scholar
  14. 14.
    Ghram N, Allani C, Oudali B, Fitouri Z, Ben Becher S. Sydenham’s chorea in children. Arch Pediatr 1999;6:1048–1052.PubMedCrossRefGoogle Scholar
  15. 15.
    Nausieda PA, Grossman BJ, Koller WC, Weiner WJ, Klawans HL. Sydenham chorea: an update. Neurology 1980;30:331–334.PubMedGoogle Scholar
  16. 16.
    Al-Eissa A. Sydenham’s chorea: a new look at an old disease. Br J Clin Pract 1993;47:14–16.PubMedGoogle Scholar
  17. 17.
    Genel F, Arslanoglu S, Uran N, Saylan B. Sydenham’s chorea: clinical findings and comparison of the efficacies of sodium valproate and carbamazepine regimens. Brain Dev 2002;24:73–76PubMedCrossRefGoogle Scholar
  18. 18.
    Asbahr FR, Negrao AB, Gentil V, et al. Obsessive-compulsive and related symptoms in children and adolescents with rheumatic fever with and without chorea: a prospective 6-month study. Am J Psychiatry 1998;155:1122–1124.PubMedGoogle Scholar
  19. 19.
    Mercadante MT, Busatto GF, Lombroso PJ, et al. The psychiatric symptoms of rheumatic fever. Am J Psychiatry 2000;157:2036–2038.PubMedCrossRefGoogle Scholar
  20. 20.
    Cardoso F, Vargas AP, Oliveira LD, Guerra AA, Amaral SV. Persistent Sydenham’s chorea. Mov Disord 1999;14:805–807.PubMedCrossRefGoogle Scholar
  21. 21.
    Giedd JN, Rapoport JL, Kruesi MJ, et al. Sydenham’s chorea: magnetic resonance imaging of the basal ganglia. Neurology 1995;45:2199–2202.PubMedGoogle Scholar
  22. 22.
    Castillo M, Kwock L, Arbelaez A. Sydenham’s chorea: MRI and proton spectroscopy. Neuroradiology 1999;41:943–945.PubMedCrossRefGoogle Scholar
  23. 23.
    Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG. Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain 2000;123:2407–2422.PubMedCrossRefGoogle Scholar
  24. 24.
    Leuzzi V, Bertini E, De Negri AM, Gallucci M, Garavaglia B. Bilateral striatal necrosis, dystonia and optic atrophy in two siblings. J Neurol Neurosurg Psychiatry 1992;55:16–19.PubMedGoogle Scholar
  25. 25.
    Giedd JN, Rapoport JL, Garvey MA, Perlmutter S, Swedo SE. MRI assessment of children with obsessive-compulsive disorder or tics associated with streptococcal infection. Am J Psychiatry 2000;157:281–283.PubMedCrossRefGoogle Scholar
  26. 26.
    Barsottini OGP, Ferraz HB, Seviliano MM, Barbieri A. Brain SPECT imaging in Sydenham’s chorea. Braz J Med Biol Res 2002;35:431–436.PubMedCrossRefGoogle Scholar
  27. 27.
    Husby G, van de Rijn I, Zabriskie JB, Abdin ZH, Williams RC Jr. Antibodies reacting with cytoplasm of subthalamic and caudate nuclei neurons in chorea and acute rheumatic fever. J Exp Med 1976;144:1094–1110.PubMedCrossRefGoogle Scholar
  28. 28.
    Kotby AA, El Badawy N, El Sokkary S, Moawad H, El Shawarby M. Antineuronal antibodies in rheumatic chorea. Clin Diagn Lab Immunol 1998;5:836–839.PubMedGoogle Scholar
  29. 29.
    Church AJ, Cardoso F, Dale RC, Lees AJ, Thompson EJ, Giovannoni G. Anti-basal ganglia antibodies in acute and persistent Sydenham’s chorea. Neurology 2002;59:227–231.PubMedGoogle Scholar
  30. 30.
    Church AJ, Dale RC, Lees AJ, Giovannoni G, Robertson MM. Tourette’s syndrome: a cross sectional study to examine the PANDAS hypothesis. J Neurol Neurosurg Psychiatry 2003;74:602–607.PubMedCrossRefGoogle Scholar
  31. 31.
    Green LN. Corticosteroids in the treatment of Sydenham’s chorea. Arch Neurol 1978;35:53–54.PubMedGoogle Scholar
  32. 32.
    Perlmutter SJ, Leitman SF, Garvey MA, et al. Therapeutic plasma exchange and intravenous immunoglobulin for obsessive-compulsive disorder and tic disorders in childhood. Lancet 1999;354:1153–1158.PubMedCrossRefGoogle Scholar
  33. 33.
    Taylor JR, Morshed SA, Parveen S, et al. An animal model of Tourette’s syndrome. Am J Psychiatry 2002;159:657–660.PubMedCrossRefGoogle Scholar
  34. 34.
    Swedo SE. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS). Mol Psychiatry 2002;7:S24–S25.PubMedCrossRefGoogle Scholar
  35. 35.
    Khanna AK, Buskirk DR, Williams RC Jr, et al. Presence of a non-HLA B cell antigen in rheumatic fever patients and their families as defined by a monoclonal antibody. J Clin Invest 1989;83:1710–1716.PubMedGoogle Scholar
  36. 36.
    Swedo SE, Leonard HL, Mittleman BB, et al. Identification of children with pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections by a marker associated with rheumatic fever. Am J Psychiatry 1997;154:110–112.PubMedGoogle Scholar
  37. 37.
    Kurlan R. Tourette’s syndrome and ‘PANDAS’: will the relation bear out? Neurology 1998;50:1530–1534.PubMedGoogle Scholar
  38. 38.
    Daoud AS, Zaki M, Shakir R, Al-Saleh Q. Effectiveness of sodium valproate in the treatment of Sydenham’s chorea. Neurology 1990;40:1140–1141.PubMedGoogle Scholar
  39. 39.
    Pena J, Mora E, Cardozo J, Molina O, Montiel C. Comparison of the efficacy of carbamazepine, haloperidol and valproic acid in the treatment of children with Sydenham’s chorea: clinical follow-up of 18 patients. Arq Neuropsiquiatr 2002;60:374–377.PubMedGoogle Scholar
  40. 40.
    Roig M, Montserrat L, Gallart A. Carbamazepine: an alternative drug for the treatment of nonhereditary chorea. Pediatrics 1988;82:492–495.PubMedGoogle Scholar
  41. 41.
    Harel L, Zecharia A, Straussberg R, Volovitz B, Amir J. Successful treatment of rheumatic chorea with carbamazepine. Pediatr Neurol 2000;23:147–151.PubMedCrossRefGoogle Scholar
  42. 42.
    Harries-Jones R, Gibson JG. Successful treatment of refractory Sydenham’s chorea with pimozide. J Neurol Neurosurg Psychiatry 1985;48:390.PubMedCrossRefGoogle Scholar
  43. 43.
    Shannon KM, Fenichel GM. Pimozide treatment of Sydenham’s chorea. Neurology 1990;40:186.PubMedGoogle Scholar
  44. 44.
    Stollerman GH. Rheumatic fever in the 21st century. Clin Infect Dis 2001;33:806–814.PubMedCrossRefGoogle Scholar
  45. 45.
    Murphy ML, Pichichero ME. Prospective identification and treatment of children with pediatric autoimmune neuropsychiatric disorder associated with group A streptococcal infection (PANDAS). Arch Pediatr Adolesc Med 2002;156:356–361.PubMedGoogle Scholar
  46. 46.
    Garvey MA, Perlmutter SJ, Allen AJ, et al. A pilot study of penicillin prophylaxis for neuropsychiatric exacerbations triggered by streptococcal infections. Biol Psychiatry 1999;45:1564–1571.PubMedCrossRefGoogle Scholar

Copyright information

© Humana Press Inc. 2005

Authors and Affiliations

  • Roser Pons
    • 1
  1. 1.Department of NeurologyColumbia University Medical CenterNew York

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