Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Overview of chorea

Oksana Suchowersky, MD, FRCPC, FCCMG
Manon Bouchard, MD, FRCPC
Section Editor
Howard I Hurtig, MD
Deputy Editor
John F Dashe, MD, PhD


The word chorea is derived from the Latin choreus, meaning "dance." Chorea is a hyperkinetic movement disorder characterized by involuntary brief, random, and irregular contractions conveying a feeling of restlessness to the observer [1]. Chorea may be caused by hereditary neurodegenerative diseases, follow structural damage to deep brain structures, or be associated with autoimmune disorders, metabolic derangement, or certain drugs and hormones. Investigation is oriented at searching for a secondary or reversible cause of chorea or confirming a hereditary condition with genetic testing. Although the chorea arriving from acquired conditions may be reversed, there is usually no specific therapy for hereditary neurodegenerative disorders. Symptomatic treatment can reduce abnormal movements regardless of the cause.

This topic will provide an overview of the various types of chorea.


Chorea, athetosis, and ballism frequently coexist in the same patient and are felt to be part of the same choreiform spectrum [2,3].

Chorea is a hyperkinetic movement disorder characterized by rapid and unpredictable contractions affecting mostly distal limbs, but also the face and trunk. The movements are involuntary and nonpatterned with variable speed, timing, and direction, flowing from one body part to another and giving, in less severe cases, an appearance of fidgetiness. The unpredictable nature of chorea is a feature that distinguishes it from tremor and dystonia [1].

Athetosis refers to slower, writhing movements with a sinuous quality. The term choreoathetosis is used when typical choreic movements coexist with athetosis.

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Nov 22, 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 ©2017 UpToDate, Inc.
  1. Cardoso F, Seppi K, Mair KJ, et al. Seminar on choreas. Lancet Neurol 2006; 5:589.
  2. Bhidayasiri R, Truong DD. Chorea and related disorders. Postgrad Med J 2004; 80:527.
  3. Suchowersky O, Muthipeedika J. A case of late-onset chorea. Nat Clin Pract Neurol 2005; 1:113.
  4. Shannon KM. Treatment of chorea. Continuum 2007; 13:72.
  5. Lee MS, Marsden CD. Movement disorders following lesions of the thalamus or subthalamic region. Mov Disord 1994; 9:493.
  6. Piccolo I, Defanti CA, Soliveri P, et al. Cause and course in a series of patients with sporadic chorea. J Neurol 2003; 250:429.
  7. Zomorrodi A, Wald ER. Sydenham's chorea in western Pennsylvania. Pediatrics 2006; 117:e675.
  8. Hensman Moss DJ, Poulter M, Beck J, et al. C9orf72 expansions are the most common genetic cause of Huntington disease phenocopies. Neurology 2014; 82:292.
  9. Schneider SA, Walker RH, Bhatia KP. The Huntington's disease-like syndromes: what to consider in patients with a negative Huntington's disease gene test. Nat Clin Pract Neurol 2007; 3:517.
  10. Gövert F, Schneider SA. Huntington's disease and Huntington's disease-like syndromes: an overview. Curr Opin Neurol 2013; 26:420.
  11. Xiang F, Almqvist EW, Huq M, et al. A Huntington disease-like neurodegenerative disorder maps to chromosome 20p. Am J Hum Genet 1998; 63:1431.
  12. Margolis RL, Holmes SE, Rosenblatt A, et al. Huntington's Disease-like 2 (HDL2) in North America and Japan. Ann Neurol 2004; 56:670.
  13. Wild EJ, Mudanohwo EE, Sweeney MG, et al. Huntington's disease phenocopies are clinically and genetically heterogeneous. Mov Disord 2008; 23:716.
  14. Santos C, Wanderley H, Vedolin L, et al. Huntington disease-like 2: the first patient with apparent European ancestry. Clin Genet 2008; 73:480.
  15. Walker RH, Jung HH, Dobson-Stone C, et al. Neurologic phenotypes associated with acanthocytosis. Neurology 2007; 68:92.
  16. Walker RH, Rasmussen A, Rudnicki D, et al. Huntington's disease--like 2 can present as chorea-acanthocytosis. Neurology 2003; 61:1002.
  17. Kambouris M, Bohlega S, Al-Tahan A, Meyer BF. Localization of the gene for a novel autosomal recessive neurodegenerative Huntington-like disorder to 4p15.3. Am J Hum Genet 2000; 66:445.
  18. Wild EJ, Tabrizi SJ. Huntington's disease phenocopy syndromes. Curr Opin Neurol 2007; 20:681.
  19. Stevanin G, Brice A. Spinocerebellar ataxia 17 (SCA17) and Huntington's disease-like 4 (HDL4). Cerebellum 2008; 7:170.
  20. Devos D, Tchofo PJ, Vuillaume I, et al. Clinical features and natural history of neuroferritinopathy caused by the 458dupA FTL mutation. Brain 2009; 132:e109.
  21. Breedveld GJ, van Dongen JW, Danesino C, et al. Mutations in TITF-1 are associated with benign hereditary chorea. Hum Mol Genet 2002; 11:971.
  22. Curtis AR, Fey C, Morris CM, et al. Mutation in the gene encoding ferritin light polypeptide causes dominant adult-onset basal ganglia disease. Nat Genet 2001; 28:350.
  23. Chinnery PF, Curtis AR, Fey C, et al. Neuroferritinopathy in a French family with late onset dominant dystonia. J Med Genet 2003; 40:e69.
  24. Maciel P, Cruz VT, Constante M, et al. Neuroferritinopathy: missense mutation in FTL causing early-onset bilateral pallidal involvement. Neurology 2005; 65:603.
  25. Batla A, Adams ME, Erro R, et al. Cortical pencil lining in neuroferritinopathy: a diagnostic clue. Neurology 2015; 84:1816.
  26. Harper PS. The epidemiology of Huntington's disease. Hum Genet 1992; 89:365.
  27. 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.
  28. Chung SJ, Im JH, Lee MC, Kim JS. Hemichorea after stroke: clinical-radiological correlation. J Neurol 2004; 251:725.
  29. Postuma RB, Lang AE. Hemiballism: revisiting a classic disorder. Lancet Neurol 2003; 2:661.
  30. Alarcón F, Zijlmans JC, Dueñas G, Cevallos N. Post-stroke movement disorders: report of 56 patients. J Neurol Neurosurg Psychiatry 2004; 75:1568.
  31. Laganiere S, Boes AD, Fox MD. Network localization of hemichorea-hemiballismus. Neurology 2016; 86:2187.
  32. Nazabal ER, Lopez JM, Perez PA, Del Corral PR. Chorea disclosing deterioration of polycythaemia vera. Postgrad Med J 2000; 76:658.
  33. O'Toole O, Lennon VA, Ahlskog JE, et al. Autoimmune chorea in adults. Neurology 2013; 80:1133.
  34. Mehta SH, Morgan JC, Sethi KD. Paraneoplastic movement disorders. Curr Neurol Neurosci Rep 2009; 9:285.
  35. Vigliani MC, Honnorat J, Antoine JC, et al. Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience. J Neurol 2011; 258:2058.
  36. Barton B, Zauber SE, Goetz CG. Movement disorders caused by medical disease. Semin Neurol 2009; 29:97.
  37. Martinková J, Valkovic P, Benetin J. Paraneoplastic chorea associated with breast cancer. Mov Disord 2009; 24:2296.
  38. Kellinghaus C, Kraus J, Blaes F, et al. CRMP-5-autoantibodies in testicular cancer associated with limbic encephalitis and choreiform dyskinesias. Eur Neurol 2007; 57:241.
  39. Lee EK, Maselli RA, Ellis WG, Agius MA. Morvan's fibrillary chorea: a paraneoplastic manifestation of thymoma. J Neurol Neurosurg Psychiatry 1998; 65:857.
  40. Arnson Y, Shoenfeld Y, Alon E, Amital H. The antiphospholipid syndrome as a neurological disease. Semin Arthritis Rheum 2010; 40:97.
  41. Benamour S, Naji T, Alaoui FZ, et al. [Neurological involvement in Behçet's disease. 154 cases from a cohort of 925 patients and review of the literature]. Rev Neurol (Paris) 2006; 162:1084.
  42. Venegas Fanchke P, Sinning M, Miranda M. Primary Sjogren's syndrome presenting as a generalized chorea. Parkinsonism Relat Disord 2005; 11:193.
  43. Duzova A, Bakkaloglu A. Central nervous system involvement in pediatric rheumatic diseases: current concepts in treatment. Curr Pharm Des 2008; 14:1295.
  44. Bushara KO. Neurologic presentation of celiac disease. Gastroenterology 2005; 128:S92.
  45. Sigal LH. The neurologic presentation of vasculitic and rheumatologic syndromes. A review. Medicine (Baltimore) 1987; 66:157.
  46. Bordelon YM, Smith M. Movement disorders in pregnancy. Semin Neurol 2007; 27:467.
  47. Chang CV, Felicio AC, Godeiro Cde O Jr, et al. Chorea-ballism as a manifestation of decompensated type 2 diabetes mellitus. Am J Med Sci 2007; 333:175.
  48. Lee BC, Hwang SH, Chang GY. Hemiballismus-hemichorea in older diabetic women: a clinical syndrome with MRI correlation. Neurology 1999; 52:646.
  49. Pacchetti C, Cristina S, Nappi G. Reversible chorea and focal dystonia in vitamin B12 deficiency. N Engl J Med 2002; 347:295.
  50. Shyambabu C, Sinha S, Taly AB, et al. Serum vitamin B12 deficiency and hyperhomocystinemia: a reversible cause of acute chorea, cerebellar ataxia in an adult with cerebral ischemia. J Neurol Sci 2008; 273:152.
  51. Edvardsson B, Persson S. Chorea associated with vitamin B12 deficiency. Eur J Neurol 2011; 18:e138.
  52. Ristić AJ, Svetel M, Dragasević N, et al. Bilateral chorea-ballism associated with hyperthyroidism. Mov Disord 2004; 19:982.
  53. Yu JH, Weng YM. Acute chorea as a presentation of Graves disease: case report and review. Am J Emerg Med 2009; 27:369.e1.
  54. Docherty MJ, Burn DJ. Hyperthyroid chorea. Handb Clin Neurol 2011; 100:279.
  55. Topakian R, Stieglbauer K, Rotaru J, et al. Hypocalcemic choreoathetosis and tetany after bisphosphonate treatment. Mov Disord 2006; 21:2026.
  56. Thomas R, Behari M, Gaikwad SB, Prasad K. An unusual case of paroxysmal kinesigenic dyskinesia. J Clin Neurosci 2002; 9:94.
  57. Rao YP, Naidu KS, Murthy JM. Idiopathic hypoparathyroidism with extensive intracranial calcification associated with kinesigenic choreoathetosis. J Assoc Physicians India 1999; 47:928.
  58. Jog MS, Lang AE. Chronic acquired hepatocerebral degeneration: case reports and new insights. Mov Disord 1995; 10:714.
  59. Lee EJ, Park JH, Ihn Yk, et al. Acute bilateral basal ganglia lesions in diabetic uraemia: diffusion-weighted MRI. Neuroradiology 2007; 49:1009.
  60. Cardoso F. HIV-related movement disorders: epidemiology, pathogenesis and management. CNS Drugs 2002; 16:663.
  61. Gallo BV, Shulman LM, Weiner WJ, et al. HIV encephalitis presenting with severe generalized chorea. Neurology 1996; 46:1163.
  62. Miyasaki JM. Chorea caused by toxins. Handb Clin Neurol 2011; 100:335.
  63. 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.
  64. Warren JD, Firgaira F, Thompson EM, et al. The causes of sporadic and 'senile' chorea. Aust N Z J Med 1998; 28:429.
  65. Kimber TE, Thompson PD. Senile chorea. Handb Clin Neurol 2011; 100:213.
  66. Blanchet PJ, Rompré PH, Lavigne GJ, Lamarche C. Oral dyskinesia: a clinical overview. Int J Prosthodont 2005; 18:10.
  67. Blanchet PJ, Popovici R, Guitard F, et al. Pain and denture condition in edentulous orodyskinesia: comparisons with tardive dyskinesia and control subjects. Mov Disord 2008; 23:1837.
  68. Harel L, Zecharia A, Straussberg R, et al. Successful treatment of rheumatic chorea with carbamazepine. Pediatr Neurol 2000; 23:147.
  69. Chandra V, Spunt AL, Rusinowitz MS. Treatment of post-traumatic choreo-athetosis with sodium valproate. J Neurol Neurosurg Psychiatry 1983; 46:963.
  70. Giroud M, Dumas R. Valproate sodium in postanoxic choreoathetosis. J Child Neurol 1986; 1:80.
  71. Kulkarni ML. Sodium valproate controls choreoathetoid movements of kernicterus. Indian Pediatr 1992; 29:1029.
  72. Lenton RJ, Copti M, Smith RG. Hemiballismus treated with sodium valproate. Br Med J (Clin Res Ed) 1981; 283:17.
  73. Siniscalchi A, Gallelli L, Davoli A, De Sarro G. Efficacy and tolerability of topiramate in vascular generalized chorea. Ann Pharmacother 2007; 41:1915.
  74. Driver-Dunckley E, Evidente VG. Hemichorea-hemiballismus may respond to topiramate. Clin Neuropharmacol 2005; 28:142.
  75. Gatto EM, Uribe Roca C, Raina G, et al. Vascular hemichorea/hemiballism and topiramate. Mov Disord 2004; 19:836.
  76. Zesiewicz TA, Sullivan KL, Hauser RA, Sanchez-Ramos J. Open-label pilot study of levetiracetam (Keppra) for the treatment of chorea in Huntington's disease. Mov Disord 2006; 21:1998.
  77. Zesiewicz TA, Pathak A, Sullivan KL, et al. Treatment of chorea with levetiracetam. Eur J Clin Pharmacol 2006; 62:87.
  78. Tsao CY. Effective treatment with oxcarbazepine in paroxysmal kinesigenic choreoathetosis. J Child Neurol 2004; 19:300.
  79. Kothare SV, Pollack P, Kulberg AG, Ravin PD. Gabapentin treatment in a child with delayed-onset hemichorea/hemiballismus. Pediatr Neurol 2000; 22:68.
  80. Chudnow RS, Mimbela RA, Owen DB, Roach ES. Gabapentin for familial paroxysmal dystonic choreoathetosis. Neurology 1997; 49:1441.
  81. Cardoso F, Maia D, Cunningham MC, Valença G. Treatment of Sydenham chorea with corticosteroids. Mov Disord 2003; 18:1374.
  82. Paz JA, Silva CA, Marques-Dias MJ. Randomized double-blind study with prednisone in Sydenham's chorea. Pediatr Neurol 2006; 34:264.
  83. Sanna G, Bertolaccini ML, Khamashta MA. Neuropsychiatric involvement in systemic lupus erythematosus: current therapeutic approach. Curr Pharm Des 2008; 14:1261.
  84. Cervera R, Bucciarelli S, Plasín MA, et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the "CAPS Registry". J Autoimmun 2009; 32:240.
  85. Weinstein R. Therapeutic apheresis in neurological disorders: a survey of the evidence in support of current category I and II indications for therapeutic plasma exchange. J Clin Apher 2008; 23:196.
  86. Peiris JB, Boralessa H, Lionel ND. Clonazepam in the treatment of choreiform activity. Med J Aust 1976; 1:225.
  87. Goto S, Kunitoku N, Hamasaki T, et al. Abolition of postapoplectic hemichorea by Vo-complex thalamotomy: long-term follow-up study. Mov Disord 2001; 16:771.
  88. Cardoso F, Jankovic J, Grossman RG, Hamilton WJ. Outcome after stereotactic thalamotomy for dystonia and hemiballismus. Neurosurgery 1995; 36:501.
  89. Choi SJ, Lee SW, Kim MC, et al. Posteroventral pallidotomy in medically intractable postapoplectic monochorea: case report. Surg Neurol 2003; 59:486.
  90. 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.
  91. Yianni J, Nandi D, Bradley K, et al. Senile chorea treated by deep brain stimulation: a clinical, neurophysiological and functional imaging study. Mov Disord 2004; 19:597.
  92. Watarai M, Hashimoto T, Yamamoto K, et al. Pallidotomy for severe generalized chorea of juvenile-onset dentatorubral-pallidoluysian atrophy. Neurology 2003; 61:1452.
  93. Edwards TC, Zrinzo L, Limousin P, Foltynie T. Deep brain stimulation in the treatment of chorea. Mov Disord 2012; 27:357.
  94. Starr PA. Deep brain stimulation for other tremors, myoclonus, and chorea. Handb Clin Neurol 2013; 116:209.