Chorea, Ballism, and Athetosis

  • Francisco CardosoEmail author


Chorea is defined as a syndrome characterized by the continuous flow of random muscle contractions. This pattern of movement conveys a feeling of restlessness to the observer. When choreic movements are more severe, assuming a flinging, sometimes violent, character, they are called ballism. Neurophysiologic studies show that chorea and ballism share the same neuronal pattern, i.e., hypoactivity of the subthalamic nucleus and increased firing rate of the globus pallidus pars interna [26]. Athetosis describes sinuous, slow movements affecting distal limbs, particularly in the arms. There is a clear decline of the use of this term in the contemporary literature. The reason behind this tendency is the realization that athetosis is better defined as dystonia occasionally associated with some degree of chorea.


Systemic Lupus Erythematosus Cerebral Palsy Rheumatic Fever Moyamoya Disease Streptococcal Infection 
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Supplementary material

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  1. 1.
    Asherson RA, Cervera R. Unusual manifestations of the antiphospholipid syndrome. Clin Rev Allergy Immunol. 2003;25:61–78.CrossRefPubMedGoogle Scholar
  2. 2.
    Ayoub EM. Resurgence of rheumatic fever in the United States. The changing picture of a preventable illness. Postgrad Med. 1992;92:133–42.CrossRefPubMedGoogle Scholar
  3. 3.
    Barsottini OG, Ferraz HB, Seviliano MM, Barbieri A. Brain SPECT imaging in Sydenham’s chorea. Braz J Med Biol Res. 2002;35:431–6.CrossRefPubMedGoogle Scholar
  4. 4.
    Brimberg L, Benhar I, Mascaro-Blanco A, et al. Behavioral, pharmacological, and immunological abnormalities after streptococcal exposure: a novel rat model of Sydenham chorea and related neuropsychiatric disorders. Neuropsychopharmacology. 2012;37:2076–87.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Cardoso F. Chorea gravidarum. Arch Neurol. 2002;59:868–70.CrossRefPubMedGoogle Scholar
  6. 6.
    Cardoso F. HIV-related movement disorders: epidemiology, pathogenesis and management. CNS Drugs. 2002;16:663–8.CrossRefPubMedGoogle Scholar
  7. 7.
    Cardoso F, Dornas L, Cunningham M, Oliveira JT. Nerve conduction study in Sydenham’s chorea. Mov Disord. 2004:360–3. doi: 10.1002/mds.20318.
  8. 8.
    Cardoso F, Jankovic J, Grossman RG, Hamilton WJ. Outcome after stereotactic thalamotomy for dystonia and hemiballismus. Neurosurgery. 1995;36:501–7.CrossRefPubMedGoogle Scholar
  9. 9.
    Cardoso F, Maia D, Cunningham MC, Valença G. Treatment of Sydenham chorea with corticosteroids. Mov Disord. 2003;18:1374–7.CrossRefPubMedGoogle Scholar
  10. 10.
    Cardoso F, Silva CE, Mota CC. Sydenham’s chorea in 50 consecutive patients with rheumatic fever. Mov Disord. 1997;12:701–3.CrossRefPubMedGoogle Scholar
  11. 11.
    Cardoso F, Vargas AP, Oliveira LD, et al. Persistent Sydenham’s chorea. Mov Disord. 1999;14:805–7.CrossRefPubMedGoogle Scholar
  12. 12.
    Choi SJ, Lee SW, Kim MC, et al. Posteroventral pallidotomy in medically intractable postapoplectic monochorea: case report. Surg Neurol. 2003;59:486–90.CrossRefPubMedGoogle Scholar
  13. 13.
    Chu K, Kang DW, Kim DE, et al. Diffusion-weighted and gradient echo magnetic resonance findings of hemichorea-hemiballismus associated with diabetic hyperglycemia: a hyperviscosity syndrome? Arch Neurol. 2002;59:448–52.CrossRefPubMedGoogle Scholar
  14. 14.
    Church AJ, Cardoso F, Dale RC, et al. Anti-basal ganglia antibodies in acute and persistent Sydenham’s chorea. Neurology. 2002;59:227–31.CrossRefPubMedGoogle Scholar
  15. 15.
    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(1–2):149–53.CrossRefPubMedGoogle Scholar
  16. 16.
    Citak EC, Gukuyener K, Karabacak NI, et al. Functional brain imaging in Sydenham’s chorea and streptococcal tic disorders. J Child Neurol. 2004;19:387–90.CrossRefPubMedGoogle Scholar
  17. 17.
    Das KB, Harris C, Smyth DP, Cross JH. Unusual side effects of lamotrigine therapy. J Child Neurol. 2003;18:479–80.CrossRefPubMedGoogle Scholar
  18. 18.
    Dorban S, Gille M, Kessler R, et al. Chorea-athetosis in the anti-Hu syndrome. Rev Neurol (Paris). 2004;160:126–9.CrossRefGoogle Scholar
  19. 19.
    Erickson JC, Jabbari B, Difazio MP. Basal ganglia injury as a complication of the ketogenic diet. Mov Disord. 2003;18:448–51.CrossRefPubMedGoogle Scholar
  20. 20.
    Faustino PC, Terreri MT, da Rocha AJ, et al. Clinical, laboratory, psychiatric and magnetic resonance findings in patients with Sydenham’s chorea. Neuroradiology. 2003;45:456–62.CrossRefPubMedGoogle Scholar
  21. 21.
    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;24:73–6.CrossRefPubMedGoogle Scholar
  22. 22.
    Ghika-Schmid F, Ghika J, Regli F, Bogousslavsky J. Hyperkinetic movement disorders during and after acute stroke: the Lausanne Stroke Registry. J Neurol Sci. 1997;146:109–16.CrossRefPubMedGoogle Scholar
  23. 23.
    Giedd JN, Rapoport JL, Kruesi MJ, et al. Sydenham’s chorea: magnetic resonance imaging of the basal ganglia. Neurology. 1995;45:2199–202.CrossRefPubMedGoogle Scholar
  24. 24.
    Gonzalez-Alegre P, Ammache Z, Davis PH, Rodnitzky RL. Moyamoya-induced paroxysmal dyskinesia. Mov Disord. 2003;18:1051–6.CrossRefPubMedGoogle Scholar
  25. 25.
    Guidelines for diagnosis of rheumatic fever, Jones criteria, 1992 update. Special Writing Group of the Committee of Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardio-Vascular Disease of the Young of the American Heart Association. Guidelines for the diagnosis of rheumatic fever. JAMA. 1992;268:2069–73.Google Scholar
  26. 26.
    Hamani C, Saint-Cyr JA, Fraser J, et al. The subthalamic nucleus in the context of movement disorders. Brain. 2004;127:4–20.CrossRefPubMedGoogle Scholar
  27. 27.
    Harrison NA, Church A, Nisbet A, Rudge P, Giovannoni G. Late recurrences of Sydenham’s chorea are not associated with anti-basal ganglia antibodies. J Neurol Neurosurg Psychiatry. 2004;75:1478–9.CrossRefPubMedPubMedCentralGoogle Scholar
  28. 28.
    Hernandez-Latorre MA, Roig-Quilis M. The efficiency of carbamazepine in a case of post-streptococcal hemichorea. Rev Neurol (Spain). 2003;37:322–6.Google Scholar
  29. 29.
    Husby G, Van De Rijn U, Zabriskie JB, et al. Antibodies reacting with cytoplasm of subthalamic and caudate nuclei neurons in chorea and acute rheumatic fever. J Exp Med. 1976;144:1094–110.CrossRefPubMedGoogle Scholar
  30. 30.
    Jankovic J. Differential diagnosis and etiology of tics. Adv Neurol. 2001;85:15–29.PubMedGoogle Scholar
  31. 31.
    Jog MS, Lang AE. Chronic acquired hepatocerebral degeneration: case reports and insights. Mov Disord. 1995;10:714–22.CrossRefPubMedGoogle Scholar
  32. 32.
    Jordan LC, Singer HS. Sydenham chorea in children. Curr Treat Options Neurol. 2003;5:283–90.CrossRefPubMedGoogle Scholar
  33. 33.
    Kalita J, Ranjan P, Misra UK, Das BK. Hemichorea: a rare presentation of tuberculoma. J Neurol Sci. 2003;208:109–11.CrossRefPubMedGoogle Scholar
  34. 34.
    Kinirons P, Fulton A, Keoghan M, et al. Paraneoplastic limbic encephalitis (PLE) and chorea associated with CRMP-5 neuronal antibody. Neurology. 2003;61:1623–4.CrossRefPubMedGoogle Scholar
  35. 35.
    Kirvan CA, Swedo SE, Heuser JS, Cunningham MW. Mimicry and autoantibody-mediated neuronal cell signaling in Sydenham chorea. Nat Med. 2003;9(7):914–20.Google Scholar
  36. 36.
    Korn-Lubetzki I, Brand A, Steiner I. Recurrence of Sydenham chorea: implications for pathogenesis. Arch Neurol. 2004;61:1261–4.CrossRefPubMedGoogle Scholar
  37. 37.
    Krauss JK, Loher TJ, Weigel R, et al. Chronic stimulation of the globus pallidus internus for treatment of non-DYT1 generalized dystonia and choreoathetosis: 2-year follow up. J Neurosurg. 2003;98:785–92.CrossRefPubMedGoogle Scholar
  38. 38.
    Kwack C, Vuong KD, Jankovic J. Migraine headache in patients with Tourette syndrome. Arch Neurol. 2003;60:1595–8.CrossRefGoogle Scholar
  39. 39.
    Leckman JF. Tourette’s syndrome. Lancet. 2002;360:1577–86.CrossRefPubMedGoogle Scholar
  40. 40.
    Lin JJ, Chang MK. Hemiballism-hemichorea and non-ketotic hyperglycemia. J Neurol Neurosurg Psychiatry. 1994;57:748–50.CrossRefPubMedPubMedCentralGoogle Scholar
  41. 41.
    Lotan D, Benhar I, Alvarez K, et al. Behavioral and neural effects of intra-striatal infusión of anti-streptococcal antibodies in rats. Brain Behav Immun. 2014;38:249–62.CrossRefPubMedPubMedCentralGoogle Scholar
  42. 42.
    Lussier D, Cruciani RA. Choreiform movements after a single dose of methadone. J Pain Symptom Manage. 2003;26:688–91.CrossRefPubMedGoogle Scholar
  43. 43.
    Maia DP, Fonseca PG, Camargos ST, et al. Pregnancy in patients with Sydenham’s chorea. Parkinsonism Relat Disord. 2012;18:458–61.CrossRefPubMedGoogle Scholar
  44. 44.
    Maia DP, Teixeira Jr AL, Cunningham MCQ, Cardoso F. Obsessive compulsive behavior, hyperactivity and attention deficit disorder in Sydenham’s chorea. Neurology. 2005;64:1799–801.CrossRefPubMedGoogle Scholar
  45. 45.
    Martinelli P, Rizzo G, Scaglione C, Capellari S. Neurosyphilis orofacial dyskinesia: the candy sign. Mov Disord. 2013;28:246–7.CrossRefPubMedGoogle Scholar
  46. 46.
    McKee D, Talbot P. Chorea as a presenting feature of variant Creutzfeldt-Jakob disease. Mov Disord. 2003;18:837–8.CrossRefPubMedGoogle Scholar
  47. 47.
    Mercadante MT, Campos MC, Marques-Dias MJ, et al. Vocal tics in Sydenham’s chorea. J Am Acad Child Adolesc Psychiatry. 1997;36:305–6.CrossRefPubMedGoogle Scholar
  48. 48.
    Miranda M, Cardoso F, Giovannoni G, Church A. Oral contraceptive induced chorea: another condition associated with anti-basal ganglia antibodies. J Neurol Neurosurg Psychiatry. 2004;75:327–8.PubMedPubMedCentralGoogle Scholar
  49. 49.
    Morris JG, Grattan-Smith P, Jankelowitz SK, et al. Athetosis II: the syndrome of mild athetoid cerebral palsy. Mov Disord. 2002;17:1281–7.CrossRefPubMedGoogle Scholar
  50. 50.
    Nausieda PA, Grossman BJ, Koller WC, et al. Sydenham’s chorea: an update. Neurology. 1980;30:331–4.CrossRefPubMedGoogle Scholar
  51. 51.
    O’Toole O, Lennon VA, Ahlskog JE, et al. Autoimmune chorea in adults. Neurology. 2013;80:1133–44.CrossRefPubMedPubMedCentralGoogle Scholar
  52. 52.
    Piccolo I, Defanti CA, Soliveri P, et al. Cause and course in a series of patients with sporadic chorea. J Neurol. 2003;250:429–35.CrossRefPubMedGoogle Scholar
  53. 53.
    Quinn N, Schrag A. Huntington’s disease and other choreas. J Neurol. 1998;245:709–16.CrossRefPubMedGoogle Scholar
  54. 54.
    Ristic AJ, Svetel M, Dragasevic N, et al. Bilateral chorea-ballism associated with hyperthyroidism. Mov Disord. 2004;19:982–3.CrossRefPubMedGoogle Scholar
  55. 55.
    Ryan M, Antony JH, Grattan-Smith PJ. Sydenham chorea: a resurgence of the 1990s? J Paediatr Child Health. 2000;36:95–6.CrossRefPubMedGoogle Scholar
  56. 56.
    Sanna G, Bertolaccini ML, Cuadrado MJ, et al. Neuropsychiatric manifestations in systemic lupus erythematosus: prevalence and association with antiphospholipid antibodies. J Rheumatol. 2003;30:985–92.PubMedGoogle Scholar
  57. 57.
    Sharp FR, Rando TA, Greenberg SA, et al. Pseudochoreoathetosis. Movements associated with loss of proprioception. Arch Neurol. 1994;51:1103–9.CrossRefPubMedGoogle Scholar
  58. 58.
    Stemper B, Thurauf N, Neundorfer B, Heckmann JG. Choreoathetosis related to lithium intoxication. Eur J Neurol. 2003;10:743–4.CrossRefPubMedGoogle Scholar
  59. 59.
    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;155:264–71.PubMedGoogle Scholar
  60. 60.
    Tani LY, Veasy LG, Minich LL, Shaddy RE. Rheumatic fever in children younger than 5 years: is the presentation different? Pediatrics. 2003;112:1065–8.CrossRefPubMedGoogle Scholar
  61. 61.
    Teixeira Jr AL, Cardoso F, Maia DP, Cunningham MC. Sydenham’s chorea may be a risk factor for drug induced parkinsonism. J Neurol Neurosurg Psychiatry. 2003;74:1350–1.CrossRefPubMedPubMedCentralGoogle Scholar
  62. 62.
    Teixeira Jr AL, Cardoso F, Souza ALS, Teixeira MM. Increased serum concentrations of monokine induced by interferon-y/CXCL9 and interferon-y-inducible protein 10/CXCL-10 in Sydenham’s chorea patients. J Neuroimmunol. 2004;150:157–62.CrossRefPubMedGoogle Scholar
  63. 63.
    Teixeira-Jr AL, Guimarães MM, Romano-Silva MA, Cardoso F. Serum from Sydenham’s chorea patients modifies intracellular calcium levels in PC12 cells by a complement independent mechanism. Mov Disord. 2005;20:843–5.CrossRefGoogle Scholar
  64. 64.
    Teixeira Jr AL, Maia DP, Cardoso F. UFMG Sydenham’s chorea rating scale (USCRS): reliability and consistency. Mov Disord. 2005:585–91. doi: 10.1002/mds.20377.
  65. 65.
    Teixeira Jr AL, Maia DP, Cardoso F. Treatment of acute Sydenham’s chorea with methyl-prednisolone pulse-therapy. Parkinsonism Relat Disord. 2005;11:327–30.Google Scholar
  66. 66.
    Teixeira Jr AL, Meira FCA, Maia DP, Cunningham MC, Cardoso F. Migraine headache in patients with Sydenham’s chorea. Cephalalgia. 2005;25:542–4.CrossRefPubMedGoogle Scholar
  67. 67.
    Thobois S, Bozio A, Ninet J, Akhavi A, Broussolle E. Chorea after cardiopulmonary bypass. Eur Neurol. 2004;51:46–7.CrossRefPubMedGoogle Scholar
  68. 68.
    Unterman A, Nolte JE, Boaz M, et al. Neuropsychiatric syndromes in systemic lupus erythematosus: a meta-analysis. Semin Arthritis Rheum. 2011;41:1–11.CrossRefPubMedGoogle Scholar
  69. 69.
    Weindl A, Kuwert T, Leenders KL, et al. Increased striatal glucose consumption in Sydenham’s chorea. Mov Disord. 1993;8:437–44.CrossRefPubMedGoogle Scholar

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© Springer-Verlag Wien 2017

Authors and Affiliations

  1. 1.Movement Disorders Unit, Neurology Service, Internal Medicine DepartmentFaculty of Medicine, Federal University of Minas GeriasBelo HorizonteBrazil

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