Skip to main content

Chorea, Ballism, and Athetosis

  • Chapter
  • First Online:
Movement Disorders Curricula

Abstract

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.

This chapter provides an overview of the most common causes of chorea in clinical practice with emphasis on clinical features, differential diagnosis, and management. The focus will be on nongenetic causes of chorea since there is one chapter on Huntington’s disease.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 149.00
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 149.00
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Asherson RA, Cervera R. Unusual manifestations of the antiphospholipid syndrome. Clin Rev Allergy Immunol. 2003;25:61–78.

    Article  PubMed  Google Scholar 

  2. Ayoub EM. Resurgence of rheumatic fever in the United States. The changing picture of a preventable illness. Postgrad Med. 1992;92:133–42.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Cardoso F. Chorea gravidarum. Arch Neurol. 2002;59:868–70.

    Article  PubMed  Google Scholar 

  6. Cardoso F. HIV-related movement disorders: epidemiology, pathogenesis and management. CNS Drugs. 2002;16:663–8.

    Article  PubMed  Google Scholar 

  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. Cardoso F, Jankovic J, Grossman RG, Hamilton WJ. Outcome after stereotactic thalamotomy for dystonia and hemiballismus. Neurosurgery. 1995;36:501–7.

    Article  CAS  PubMed  Google Scholar 

  9. Cardoso F, Maia D, Cunningham MC, Valença G. Treatment of Sydenham chorea with corticosteroids. Mov Disord. 2003;18:1374–7.

    Article  PubMed  Google Scholar 

  10. Cardoso F, Silva CE, Mota CC. Sydenham’s chorea in 50 consecutive patients with rheumatic fever. Mov Disord. 1997;12:701–3.

    Article  CAS  PubMed  Google Scholar 

  11. Cardoso F, Vargas AP, Oliveira LD, et al. Persistent Sydenham’s chorea. Mov Disord. 1999;14:805–7.

    Article  CAS  PubMed  Google Scholar 

  12. Choi SJ, Lee SW, Kim MC, et al. Posteroventral pallidotomy in medically intractable postapoplectic monochorea: case report. Surg Neurol. 2003;59:486–90.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  17. Das KB, Harris C, Smyth DP, Cross JH. Unusual side effects of lamotrigine therapy. J Child Neurol. 2003;18:479–80.

    Article  PubMed  Google Scholar 

  18. Dorban S, Gille M, Kessler R, et al. Chorea-athetosis in the anti-Hu syndrome. Rev Neurol (Paris). 2004;160:126–9.

    Article  CAS  Google Scholar 

  19. Erickson JC, Jabbari B, Difazio MP. Basal ganglia injury as a complication of the ketogenic diet. Mov Disord. 2003;18:448–51.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  23. Giedd JN, Rapoport JL, Kruesi MJ, et al. Sydenham’s chorea: magnetic resonance imaging of the basal ganglia. Neurology. 1995;45:2199–202.

    Article  CAS  PubMed  Google Scholar 

  24. Gonzalez-Alegre P, Ammache Z, Davis PH, Rodnitzky RL. Moyamoya-induced paroxysmal dyskinesia. Mov Disord. 2003;18:1051–6.

    Article  PubMed  Google Scholar 

  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. Hamani C, Saint-Cyr JA, Fraser J, et al. The subthalamic nucleus in the context of movement disorders. Brain. 2004;127:4–20.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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.

    CAS  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  30. Jankovic J. Differential diagnosis and etiology of tics. Adv Neurol. 2001;85:15–29.

    CAS  PubMed  Google Scholar 

  31. Jog MS, Lang AE. Chronic acquired hepatocerebral degeneration: case reports and insights. Mov Disord. 1995;10:714–22.

    Article  CAS  PubMed  Google Scholar 

  32. Jordan LC, Singer HS. Sydenham chorea in children. Curr Treat Options Neurol. 2003;5:283–90.

    Article  PubMed  Google Scholar 

  33. Kalita J, Ranjan P, Misra UK, Das BK. Hemichorea: a rare presentation of tuberculoma. J Neurol Sci. 2003;208:109–11.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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. Korn-Lubetzki I, Brand A, Steiner I. Recurrence of Sydenham chorea: implications for pathogenesis. Arch Neurol. 2004;61:1261–4.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  38. Kwack C, Vuong KD, Jankovic J. Migraine headache in patients with Tourette syndrome. Arch Neurol. 2003;60:1595–8.

    Article  Google Scholar 

  39. Leckman JF. Tourette’s syndrome. Lancet. 2002;360:1577–86.

    Article  PubMed  Google Scholar 

  40. Lin JJ, Chang MK. Hemiballism-hemichorea and non-ketotic hyperglycemia. J Neurol Neurosurg Psychiatry. 1994;57:748–50.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  42. Lussier D, Cruciani RA. Choreiform movements after a single dose of methadone. J Pain Symptom Manage. 2003;26:688–91.

    Article  PubMed  Google Scholar 

  43. Maia DP, Fonseca PG, Camargos ST, et al. Pregnancy in patients with Sydenham’s chorea. Parkinsonism Relat Disord. 2012;18:458–61.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  45. Martinelli P, Rizzo G, Scaglione C, Capellari S. Neurosyphilis orofacial dyskinesia: the candy sign. Mov Disord. 2013;28:246–7.

    Article  PubMed  Google Scholar 

  46. McKee D, Talbot P. Chorea as a presenting feature of variant Creutzfeldt-Jakob disease. Mov Disord. 2003;18:837–8.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  PubMed Central  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  50. Nausieda PA, Grossman BJ, Koller WC, et al. Sydenham’s chorea: an update. Neurology. 1980;30:331–4.

    Article  CAS  PubMed  Google Scholar 

  51. O’Toole O, Lennon VA, Ahlskog JE, et al. Autoimmune chorea in adults. Neurology. 2013;80:1133–44.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  53. Quinn N, Schrag A. Huntington’s disease and other choreas. J Neurol. 1998;245:709–16.

    Article  CAS  PubMed  Google Scholar 

  54. Ristic AJ, Svetel M, Dragasevic N, et al. Bilateral chorea-ballism associated with hyperthyroidism. Mov Disord. 2004;19:982–3.

    Article  PubMed  Google Scholar 

  55. Ryan M, Antony JH, Grattan-Smith PJ. Sydenham chorea: a resurgence of the 1990s? J Paediatr Child Health. 2000;36:95–6.

    Article  CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  57. Sharp FR, Rando TA, Greenberg SA, et al. Pseudochoreoathetosis. Movements associated with loss of proprioception. Arch Neurol. 1994;51:1103–9.

    Article  CAS  PubMed  Google Scholar 

  58. Stemper B, Thurauf N, Neundorfer B, Heckmann JG. Choreoathetosis related to lithium intoxication. Eur J Neurol. 2003;10:743–4.

    Article  CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  Google Scholar 

  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. 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. Teixeira Jr AL, Meira FCA, Maia DP, Cunningham MC, Cardoso F. Migraine headache in patients with Sydenham’s chorea. Cephalalgia. 2005;25:542–4.

    Article  PubMed  Google Scholar 

  67. Thobois S, Bozio A, Ninet J, Akhavi A, Broussolle E. Chorea after cardiopulmonary bypass. Eur Neurol. 2004;51:46–7.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  69. Weindl A, Kuwert T, Leenders KL, et al. Increased striatal glucose consumption in Sydenham’s chorea. Mov Disord. 1993;8:437–44.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Francisco Cardoso MD, PhD, FAAN .

Editor information

Editors and Affiliations

1 Electronic Supplementary Material

Below is the link to the electronic supplementary material.

(MOV 14416 kb)

(MOV 6619 kb)

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer-Verlag Wien

About this chapter

Cite this chapter

Cardoso, F. (2017). Chorea, Ballism, and Athetosis. In: Falup-Pecurariu, C., Ferreira, J., Martinez-Martin, P., Chaudhuri, K. (eds) Movement Disorders Curricula. Springer, Vienna. https://doi.org/10.1007/978-3-7091-1628-9_26

Download citation

  • DOI: https://doi.org/10.1007/978-3-7091-1628-9_26

  • Published:

  • Publisher Name: Springer, Vienna

  • Print ISBN: 978-3-7091-1627-2

  • Online ISBN: 978-3-7091-1628-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics