UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Sydenham chorea

Author
Donald L Gilbert, MD, MS
Section Editors
Marc C Patterson, MD, FRACP
Sheldon L Kaplan, MD
Thomas JA Lehman, MD
Deputy Editor
Carrie Armsby, MD, MPH

INTRODUCTION

Sydenham chorea (SC), or rheumatic chorea, is one of the major clinical manifestations of acute rheumatic fever (ARF) and is the most common form of acquired chorea in childhood. It is a movement disorder characterized by chorea (involuntary brief, random and irregular movements of the limbs and face), emotional lability, and hypotonia.

This topic reviews the clinical manifestations, diagnosis, and treatment of SC. Other clinical manifestations of ARF and the approach to diagnosis and treatment of rheumatic fever are presented separately. (See "Acute rheumatic fever: Clinical manifestations and diagnosis" and "Acute rheumatic fever: Treatment and prevention".)

PATHOPHYSIOLOGY

Although SC is clearly related to group A streptococcal infection [1], its pathogenesis is not completely understood. Molecular mimicry, in which antibodies directed against part of the group A streptococcus bacterium crossreact with host antigens in susceptible subjects, is thought to play an important role.

In acute rheumatic fever (ARF), antibodies are mounted against N-acetyl-beta-D-glucosamine (NABG or GlcNAc), the immunodominant carbohydrate antigen of group A streptococci. These antibodies likely play a role in valvular injury in rheumatic carditis, and in other manifestations of ARF (see "Acute rheumatic fever: Epidemiology and pathogenesis", section on 'Molecular mimicry').

Different subsets of NABG antibodies appear to correlate with distinct clinical manifestations of ARF. In SC, the antibodies bind to lysoganglioside on the neuronal cell surface [2,3], where they are capable of triggering a signaling cascade [4]. These antibodies also recognize the intracellular protein tubulin [5]. Tubulin-specific antibodies are not found in patients with ARF without SC, or in patients who have recovered from SC. The genes encoding these antibodies are similar to the genes encoding antibodies implicated in the pathogenesis of motor neuropathies [6]. Thus, tubulin appears to be an important neuronal target in the pathogenesis of SC.

                       

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Aug 2016. | This topic last updated: Sep 2, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
References
Top
  1. TARANTA A, STOLLERMAN GH. The relationship of Sydenham's chorea to infection with group A streptococci. Am J Med 1956; 20:170.
  2. Husby G, van de Rijn I, Zabriskie JB, et al. Antibodies reacting with cytoplasm of subthalamic and caudate nuclei neurons in chorea and acute rheumatic fever. J Exp Med 1976; 144:1094.
  3. Kotby AA, El Badawy N, El Sokkary S, et al. Antineuronal antibodies in rheumatic chorea. Clin Diagn Lab Immunol 1998; 5:836.
  4. Kirvan CA, Swedo SE, Heuser JS, Cunningham MW. Mimicry and autoantibody-mediated neuronal cell signaling in Sydenham chorea. Nat Med 2003; 9:914.
  5. Kirvan CA, Cox CJ, Swedo SE, Cunningham MW. Tubulin is a neuronal target of autoantibodies in Sydenham's chorea. J Immunol 2007; 178:7412.
  6. Weng NP, Yu-Lee LY, Sanz I, et al. Structure and specificities of anti-ganglioside autoantibodies associated with motor neuropathies. J Immunol 1992; 149:2518.
  7. Giedd JN, Rapoport JL, Kruesi MJ, et al. Sydenham's chorea: magnetic resonance imaging of the basal ganglia. Neurology 1995; 45:2199.
  8. Traill Z, Pike M, Byrne J. Sydenham's chorea: a case showing reversible striatal abnormalities on CT and MRI. Dev Med Child Neurol 1995; 37:270.
  9. Lee PH, Nam HS, Lee KY, et al. Serial brain SPECT images in a case of Sydenham chorea. Arch Neurol 1999; 56:237.
  10. Aron AM. Sydenham's chorea: positron emission tomographic (PET) scan studies. J Child Neurol 2005; 20:832.
  11. Eshel G, Lahat E, Azizi E, et al. Chorea as a manifestation of rheumatic fever--a 30-year survey (1960-1990). Eur J Pediatr 1993; 152:645.
  12. Seckeler MD, Hoke TR. The worldwide epidemiology of acute rheumatic fever and rheumatic heart disease. Clin Epidemiol 2011; 3:67.
  13. Veasy LG, Tani LY, Hill HR. Persistence of acute rheumatic fever in the intermountain area of the United States. J Pediatr 1994; 124:9.
  14. Centers for Disease Control (CDC). Acute rheumatic fever--Utah. MMWR Morb Mortal Wkly Rep 1987; 36:108.
  15. Stollerman GH. Rheumatic fever. Lancet 1997; 349:935.
  16. Carapetis JR, Currie BJ. Rheumatic chorea in northern Australia: a clinical and epidemiological study. Arch Dis Child 1999; 80:353.
  17. Zomorrodi A, Wald ER. Sydenham's chorea in western Pennsylvania. Pediatrics 2006; 117:e675.
  18. Demiroren K, Yavuz H, Cam L, et al. Sydenham's chorea: a clinical follow-up of 65 patients. J Child Neurol 2007; 22:550.
  19. ARON AM, FREEMAN JM, CARTER S. THE NATURAL HISTORY OF SYDENHAM'S CHOREA. REVIEW OF THE LITERATURE AND LONG-TERM EVALUATION WITH EMPHASIS ON CARDIAC SEQUELAE. Am J Med 1965; 38:83.
  20. Cardoso F, Vargas AP, Oliveira LD, et al. Persistent Sydenham's chorea. Mov Disord 1999; 14:805.
  21. Kulkarni ML, Anees S. Sydenham's chorea. Indian Pediatr 1996; 33:112.
  22. Harsányi E, Moreira J, Kummer A, et al. Language Impairment in Adolescents With Sydenham Chorea. Pediatr Neurol 2015; 53:412.
  23. Gordon W. A Note on the Knee-Jerk in Chorea. Br Med J 1901; 1:765.
  24. Swedo SE, Leonard HL, Schapiro MB, et al. Sydenham's chorea: physical and psychological symptoms of St Vitus dance. Pediatrics 1993; 91:706.
  25. Maia DP, Teixeira AL Jr, Quintão Cunningham MC, Cardoso F. Obsessive compulsive behavior, hyperactivity, and attention deficit disorder in Sydenham chorea. Neurology 2005; 64:1799.
  26. Ridel KR, Lipps TD, Gilbert DL. The prevalence of neuropsychiatric disorders in Sydenham's chorea. Pediatr Neurol 2010; 42:243.
  27. 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.
  28. Asbahr FR, Negrão 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.
  29. Punukollu M, Mushet N, Linney M, et al. Neuropsychiatric manifestations of Sydenham's chorea: a systematic review. Dev Med Child Neurol 2016; 58:16.
  30. Elevli M, Celebi A, Tombul T, Gökalp AS. Cardiac involvement in Sydenham's chorea: clinical and Doppler echocardiographic findings. Acta Paediatr 1999; 88:1074.
  31. Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation 2015; 131:1806.
  32. Johnson DR, Kurlan R, Leckman J, Kaplan EL. The human immune response to streptococcal extracellular antigens: clinical, diagnostic, and potential pathogenetic implications. Clin Infect Dis 2010; 50:481.
  33. Castelnovo G, Renard D. Magnetic resonance imaging in Sydenham chorea. Acta Neurol Belg 2012; 112:397.
  34. Robertson WC Jr, Smith CD. Sydenham's chorea in the age of MRI: a case report and review. Pediatr Neurol 2002; 27:65.
  35. Goldman S, Amrom D, Szliwowski HB, et al. Reversible striatal hypermetabolism in a case of Sydenham's chorea. Mov Disord 1993; 8:355.
  36. Oosterveer DM, Overweg-Plandsoen WC, Roos RA. Sydenham's chorea: a practical overview of the current literature. Pediatr Neurol 2010; 43:1.
  37. Axley J. Rheumatic chorea controlled with haloperidol. J Pediatr 1972; 81:1216.
  38. al-Eissa A. Sydenham's chorea: a new look at an old disease. Br J Clin Pract 1993; 47:14.
  39. Harries-Jones R, Gibson JG. Successful treatment of refractory Sydenham's chorea with pimozide. J Neurol Neurosurg Psychiatry 1985; 48:390.
  40. Harel L, Zecharia A, Straussberg R, et al. Successful treatment of rheumatic chorea with carbamazepine. Pediatr Neurol 2000; 23:147.
  41. Alvarez LA, Novak G. Valproic acid in the treatment of Sydenham chorea. Pediatr Neurol 1985; 1:317.
  42. Dhanaraj M, Radhakrishnan AR, Srinivas K, Sayeed ZA. Sodium valproate in Sydenham's chorea. Neurology 1985; 35:114.
  43. van Immerzeel TD, van Gilst RM, Hartwig NG. Beneficial use of immunoglobulins in the treatment of Sydenham chorea. Eur J Pediatr 2010; 169:1151.
  44. Walker K, Brink A, Lawrenson J, et al. Treatment of sydenham chorea with intravenous immunoglobulin. J Child Neurol 2012; 27:147.
  45. Boersma NA, Schippers H, Kuijpers T, Heidema J. Successful treatment of Sydenham's chorea with intravenous immunoglobulin. BMJ Case Rep 2016; 2016.
  46. Gwathmey K, Balogun RA, Burns T. Neurologic indications for therapeutic plasma exchange: 2013 update. J Clin Apher 2014; 29:211.
  47. Miranda M, Walker RH, Saez D, Renner V. Severe Sydenham's chorea (chorea paralytica) successfully treated with plasmapheresis. J Clin Mov Disord 2015; 2:2.
  48. Latimer ME, L'Etoile N, Seidlitz J, Swedo SE. Therapeutic plasma apheresis as a treatment for 35 severely ill children and adolescents with pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections. J Child Adolesc Psychopharmacol 2015; 25:70.
  49. Aranson N, Douglas HS, et al. Cortisone in Sydenham's chorea. JAMA 1951; 145:580.
  50. Green LN. Corticosteroids in the treatment of Sydenham's chorea. Arch Neurol 1978; 35:53.
  51. Barash J, Margalith D, Matitiau A. Corticosteroid treatment in patients with Sydenham's chorea. Pediatr Neurol 2005; 32:205.
  52. Paz JA, Silva CA, Marques-Dias MJ. Randomized double-blind study with prednisone in Sydenham's chorea. Pediatr Neurol 2006; 34:264.
  53. Thompson JA, Tani LY, Bale JF. Sydenham's chorea: the Utah experience (abstract). Ann Neurol 1999; 48:523.
  54. HITCHENS RA. Recurrent attacks of acute rheumatism in school-children. Ann Rheum Dis 1958; 17:293.
  55. LESSOF MH, BYWATERS EG. The duration of chorea. Br Med J 1956; 1:1520.
  56. Gurkas E, Karalok ZS, Taskin BD, et al. Predictors of recurrence in Sydenham's chorea: Clinical observation from a single center. Brain Dev 2016; 38:827.
  57. Korn-Lubetzki I, Brand A, Steiner I. Recurrence of Sydenham chorea: implications for pathogenesis. Arch Neurol 2004; 61:1261.
  58. Berrios X, Quesney F, Morales A, et al. Are all recurrences of "pure" Sydenham chorea true recurrences of acute rheumatic fever? J Pediatr 1985; 107:867.
  59. Church AJ, Dale RC, Cardoso F, et al. CSF and serum immune parameters in Sydenham's chorea: evidence of an autoimmune syndrome? J Neuroimmunol 2003; 136:149.
  60. Church AJ, Cardoso F, Dale RC, et al. Anti-basal ganglia antibodies in acute and persistent Sydenham's chorea. Neurology 2002; 59:227.