Smarter Decisions,
Better Care

UpToDate synthesizes the most recent medical information into evidence-based practical recommendations clinicians trust to make the right point-of-care decisions.

  • Rigorous editorial process: Evidence-based treatment recommendations
  • World-Renowned physician authors: over 5,100 physician authors and editors around the globe
  • Innovative technology: integrates into the workflow; access from EMRs

Choose from the list below to learn more about subscriptions for a:


Subscribers log in here


Sydenham chorea

INTRODUCTION

Sydenham chorea (SC), also known as St. Vitus dance, St. Johannis' chorea, chorea minor, 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.

The incidence of ARF and SC has declined dramatically in the Western world [1]. However, chorea is still a common manifestation of rheumatic fever, particularly in developing countries. In the United States, chorea was reported in 18 to 36 percent of cases of rheumatic fever [2-5]. Chorea also may occur as the initial manifestation of other illnesses, including post-infectious syndromes or primary immune disorders, which include N-methyl-D-aspartate (NMDA) receptor encephalitis and systemic lupus erythematosus [6]. (See 'Differential diagnosis' below.)

Other clinical manifestations of ARF and the approach to diagnosis of rheumatic fever are presented separately. (See "Clinical manifestations and diagnosis of acute rheumatic fever".)

PATHOPHYSIOLOGY

Although SC clearly is related to group A streptococcal infection [7], 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, 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 "Epidemiology and pathogenesis of acute rheumatic fever", section on 'Molecular mimicry'). Different subsets of NABG antibodies appear to correlate with distinct clinical manifestations of ARF. In Sydenham chorea, the antibodies bind to lysoganglioside on the neuronal cell surface [8,9], where they are capable of triggering a signaling cascade [10]. These antibodies also recognize the intracellular protein tubulin [11]. Tubulin-specific antibodies are not found in patients with acute rheumatic fever 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 [12]. Thus, tubulin appears to be an important neuronal target in the pathogenesis of Sydenham chorea.

             

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 2014. | This topic last updated: Apr 29, 2014.
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 ©2014 UpToDate, Inc.
References
Top
  1. 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.
  2. Veasy LG, Tani LY, Hill HR. Persistence of acute rheumatic fever in the intermountain area of the United States. J Pediatr 1994; 124:9.
  3. Centers for Disease Control (CDC). Acute rheumatic fever--Utah. MMWR Morb Mortal Wkly Rep 1987; 36:108.
  4. Stollerman GH. Rheumatic fever. Lancet 1997; 349:935.
  5. Carapetis JR, Currie BJ. Rheumatic chorea in northern Australia: a clinical and epidemiological study. Arch Dis Child 1999; 80:353.
  6. Benseler SM, Silverman ED. Neuropsychiatric involvement in pediatric systemic lupus erythematosus. Lupus 2007; 16:564.
  7. TARANTA A, STOLLERMAN GH. The relationship of Sydenham's chorea to infection with group A streptococci. Am J Med 1956; 20:170.
  8. 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.
  9. Kotby AA, El Badawy N, El Sokkary S, et al. Antineuronal antibodies in rheumatic chorea. Clin Diagn Lab Immunol 1998; 5:836.
  10. Kirvan CA, Swedo SE, Heuser JS, Cunningham MW. Mimicry and autoantibody-mediated neuronal cell signaling in Sydenham chorea. Nat Med 2003; 9:914.
  11. Kirvan CA, Cox CJ, Swedo SE, Cunningham MW. Tubulin is a neuronal target of autoantibodies in Sydenham's chorea. J Immunol 2007; 178:7412.
  12. 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.
  13. Buchanon DN, Walker AE, Case TJ. The pathogenesis of chorea. J Pediatr 1942; 10:555.
  14. Lange H, Thörner G, Hopf A, Schröder KF. Morphometric studies of the neuropathological changes in choreatic diseases. J Neurol Sci 1976; 28:401.
  15. Giedd JN, Rapoport JL, Kruesi MJ, et al. Sydenham's chorea: magnetic resonance imaging of the basal ganglia. Neurology 1995; 45:2199.
  16. 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.
  17. Lee PH, Nam HS, Lee KY, et al. Serial brain SPECT images in a case of Sydenham chorea. Arch Neurol 1999; 56:237.
  18. Aron AM. Sydenham's chorea: positron emission tomographic (PET) scan studies. J Child Neurol 2005; 20:832.
  19. Zomorrodi A, Wald ER. Sydenham's chorea in western Pennsylvania. Pediatrics 2006; 117:e675.
  20. 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.
  21. Cardoso F, Vargas AP, Oliveira LD, et al. Persistent Sydenham's chorea. Mov Disord 1999; 14:805.
  22. Kulkarni ML, Anees S. Sydenham's chorea. Indian Pediatr 1996; 33:112.
  23. Gordon W. A Note on the Knee-Jerk in Chorea. Br Med J 1901; 1:765.
  24. Ch'ien LT, Economides AN, Lemmi H. Sydenham's chorea and seizures. Clinical and electroencephalographic studies. Arch Neurol 1978; 35:382.
  25. CHUN RW, SMITH NJ, FORSTER FM. Papilledema in Sydenham's chorea. Am J Dis Child 1961; 101:641.
  26. COHEN MG. PSEUDOTUMOR CEREBRI FOLLOWING ACUTE RHEUMATIC FEVER. Del Med J 1963; 35:257.
  27. Willetts GS. Obstruction of central retinal artery with Sydenham's chorea. Br Med J 1961; 5253:684.
  28. FREEMAN JM, ARON AM, COLLARD JE, MACKAY MC. THE EMOTIONAL CORRELATES OF SYDENHAM'S CHOREA. Pediatrics 1965; 35:42.
  29. Swedo SE, Leonard HL, Schapiro MB, et al. Sydenham's chorea: physical and psychological symptoms of St Vitus dance. Pediatrics 1993; 91:706.
  30. 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.
  31. 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.
  32. Elevli M, Celebi A, Tombul T, Gökalp AS. Cardiac involvement in Sydenham's chorea: clinical and Doppler echocardiographic findings. Acta Paediatr 1999; 88:1074.
  33. Cardoso F, Seppi K, Mair KJ, et al. Seminar on choreas. Lancet Neurol 2006; 5:589.
  34. Ayoub EM, Wannamaker LW. Streptococcal antibody titers in Sydenham's chorea. Pediatrics 1966; 38:946.
  35. HARRIS TN, FRIEDMAN S, McLEAN DC. Determination of some streptococcal antibody titers and acute phase reactants in patients with chorea. Pediatrics 1958; 21:13.
  36. Hacohen Y, Dlamini N, Hedderly T, et al. N-methyl-D-aspartate receptor antibody-associated movement disorder without encephalopathy. Dev Med Child Neurol 2014; 56:190.
  37. Armangue T, Petit-Pedrol M, Dalmau J. Autoimmune encephalitis in children. J Child Neurol 2012; 27:1460.
  38. Lin JJ, Lin KL, Chiu CH, et al. Antineuronal antibodies and infectious pathogens in severe acute pediatric encephalitis. J Child Neurol 2014; 29:11.
  39. Goldman S, Amrom D, Szliwowski HB, et al. Reversible striatal hypermetabolism in a case of Sydenham's chorea. Mov Disord 1993; 8:355.
  40. HITCHENS RA. Recurrent attacks of acute rheumatism in school-children. Ann Rheum Dis 1958; 17:293.
  41. LESSOF MH, BYWATERS EG. The duration of chorea. Br Med J 1956; 1:1520.
  42. Alvarez LA, Novak G. Valproic acid in the treatment of Sydenham chorea. Pediatr Neurol 1985; 1:317.
  43. Dhanaraj M, Radhakrishnan AR, Srinivas K, Sayeed ZA. Sodium valproate in Sydenham's chorea. Neurology 1985; 35:114.
  44. Oosterveer DM, Overweg-Plandsoen WC, Roos RA. Sydenham's chorea: a practical overview of the current literature. Pediatr Neurol 2010; 43:1.
  45. Axley J. Rheumatic chorea controlled with haloperidol. J Pediatr 1972; 81:1216.
  46. al-Eissa A. Sydenham's chorea: a new look at an old disease. Br J Clin Pract 1993; 47:14.
  47. Harries-Jones R, Gibson JG. Successful treatment of refractory Sydenham's chorea with pimozide. J Neurol Neurosurg Psychiatry 1985; 48:390.
  48. Harel L, Zecharia A, Straussberg R, et al. Successful treatment of rheumatic chorea with carbamazepine. Pediatr Neurol 2000; 23:147.
  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. Garvey MA, Snider LA, Leitman SF, et al. Treatment of Sydenham's chorea with intravenous immunoglobulin, plasma exchange, or prednisone. J Child Neurol 2005; 20:424.
  55. van Immerzeel TD, van Gilst RM, Hartwig NG. Beneficial use of immunoglobulins in the treatment of Sydenham chorea. Eur J Pediatr 2010; 169:1151.
  56. Walker K, Brink A, Lawrenson J, et al. Treatment of sydenham chorea with intravenous immunoglobulin. J Child Neurol 2012; 27:147.
  57. Golden AS, Haut SR, Moshé SL. Nonepileptic uses of antiepileptic drugs in children and adolescents. Pediatr Neurol 2006; 34:421.
  58. Korn-Lubetzki I, Brand A, Steiner I. Recurrence of Sydenham chorea: implications for pathogenesis. Arch Neurol 2004; 61:1261.
  59. 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.
  60. 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.
  61. Church AJ, Cardoso F, Dale RC, et al. Anti-basal ganglia antibodies in acute and persistent Sydenham's chorea. Neurology 2002; 59:227.