Phenomenology of Chorea

  • Steven J. FruchtEmail author
  • Pichet Termsarasab


The differential diagnosis of chorea is broad, and phenomenology of the examination offers only limited help in identifying chorea’s etiology. Many forms of chorea are treatable, and prompt diagnosis allows for prompt evaluation of chorea’s cause and appropriate therapy.


Chorea Huntington’s disease Sydenham’s chorea Neuroacanthocytosis Athetosis Hemiballism 

Supplementary material

Video 7.1

This Chapter’s video montage of chorea consists of five segments: genetic choreas; autoimmune choreas; secondary choreas; hemiballism/hemichorea; and chorea storm (MP4 5753187 kb)


  1. 1.
    Goetz CG, Chmura TA, Lanska DJ. History of chorea: part 3 of the MDS-sponsored history of movement disorders exhibit, Barcelona, June 2000. Mov Disord. 2001;16(2):331–8.CrossRefGoogle Scholar
  2. 2.
    Osler W. On chorea and choreiform affections. London: H.K. Lewis; 1894.Google Scholar
  3. 3.
    Huntington G. On chorea. Med Surg Reporter. 1872;26:317–21.Google Scholar
  4. 4.
    Lanska DJ. George Huntington (1850-1916) and hereditary chorea. J Hist Neurosci. 2000;9(1):76–89.;1-2;FT076.CrossRefPubMedGoogle Scholar
  5. 5.
    Gusella JF, Wexler NS, Conneally PM, Naylor SL, Anderson MA, Tanzi RE, et al. A polymorphic DNA marker genetically linked to Huntington’s disease. Nature. 1983;306(5940):234–8.CrossRefGoogle Scholar
  6. 6.
    Huntington’s Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington’s disease chromosomes. The Huntington’s Disease Collaborative Research Group. Cell. 1993;72(6):971–83.CrossRefGoogle Scholar
  7. 7.
    Hammond WA. Athetosis. In: A treatise on diseases of the nervous system. New York: D. Appleton and Co; 1871. p. 654–62.Google Scholar
  8. 8.
    Hammond WA. Athetosis. In: A treatise on diseases of the nervous system. 8th ed. New York: D. Appleton and Co.; 1886. p. 325–34.Google Scholar
  9. 9.
    Lanska DJ. Chapter 33: the history of movement disorders. Handb Clin Neurol. 2010;95:501–46. Scholar
  10. 10.
    Hammond GM. Pathological findings in the original case of athetosis. J Nerv Ment Dis. 1890;17:555.Google Scholar
  11. 11.
    Mitchell SW. Post-paralytic chorea. Am J Med Sci. 1874;68:342–52.CrossRefGoogle Scholar
  12. 12.
    Lanska DJ. Early controversies over athetosis: I. clinical features, differentiation from other movement disorders, associated conditions, and pathology. Tremor Other Hyperkinet Mov (N Y). 2013;3
  13. 13.
    Hammond WA, Hammond GM. Athetosis. In: A treatise on diseases of the nervous system. 9th ed. New York: D. Appleton and Co.; 1893. p. 315–25.Google Scholar
  14. 14.
    Morris JG, Grattan-Smith P, Jankelowitz SK, Fung VS, Clouston PD, Hayes MW. Athetosis II: the syndrome of mild athetoid cerebral palsy. Mov Disord. 2002;17(6):1281–7. Scholar
  15. 15.
    Sanger TD, Chen D, Fehlings DL, Hallett M, Lang AE, Mink JW, et al. Definition and classification of hyperkinetic movements in childhood. Mov Disord. 2010;25(11):1538–49. Scholar
  16. 16.
    McCusker E, Loy CT. The many facets of unawareness in Huntington disease. Tremor Other Hyperkinet Mov (N Y). 2014;4:257. Scholar
  17. 17.
    Ryan C, Ahlskog JE, Savica R. Hyperglycemic chorea/ballism ascertained over 15 years at a referral medical center. Parkinsonism Relat Disord. 2018;48:97–100. Scholar
  18. 18.
    Suri R, Rodriguez-Porcel F, Donohue K, Jesse E, Lovera L, Dwivedi AK, et al. Post-stroke movement disorders: the clinical, neuroanatomic, and demographic portrait of 284 published cases. J Stroke Cerebrovasc Dis. 2018;27(9):2388–97. Scholar
  19. 19.
    Crealey M, Allen NM, Webb D, Bouldin A, Mc Sweeney N, Peake D, et al. Sydenham’s chorea: not gone but perhaps forgotten. Arch Dis Child. 2015;100(12):1160–2. Scholar
  20. 20.
    Cardoso F. Autoimmune choreas. J Neurol Neurosurg Psychiatry. 2017;88(5):412–7. Scholar
  21. 21.
    Peall KJ, Kurian MA. Benign hereditary chorea: an update. Tremor Other Hyperkinet Mov (N Y). 2015;5:314. Scholar
  22. 22.
    Teive HAG, Camargo CHF, Munhoz RP. More than ataxia - movement disorders in ataxia-telangiectasia. Parkinsonism Relat Disord. 2018;46:3–8. Scholar
  23. 23.
    Pearson TS. More than Ataxia: hyperkinetic movement disorders in childhood autosomal recessive Ataxia syndromes. Tremor Other Hyperkinet Mov (N Y). 2016;6:368. Scholar
  24. 24.
    Baizabal-Carvallo JF, Stocco A, Muscal E, Jankovic J. The spectrum of movement disorders in children with anti-NMDA receptor encephalitis. Mov Disord. 2013;28(4):543–7. Scholar
  25. 25.
    Williams KA, Swedo SE. Post-infectious autoimmune disorders: Sydenham’s chorea, PANDAS and beyond. Brain Res. 2015;1617:144–54. Scholar
  26. 26.
    Pagan F, Torres-Yaghi Y, Altshuler M. The diagnosis and natural history of Huntington disease. Handb Clin Neurol. 2017;144:63–7. Scholar
  27. 27.
    McGarry A, Biglan KM. Preclinical motor manifestations of Huntington disease. Handb Clin Neurol. 2017;144:93–8. Scholar
  28. 28.
    Huntington Study Group PI, Biglan KM, Shoulson I, Kieburtz K, Oakes D, Kayson E, et al. Clinical-genetic associations in the prospective Huntington at Risk Observational Study (PHAROS): implications for clinical trials. JAMA Neurol. 2016;73(1):102–10. Scholar
  29. 29.
    Kumar N, Rizek P, Jog M. Neuroferritinopathy: pathophysiology, presentation, differential diagnoses and management. Tremor Other Hyperkinet Mov (N Y). 2016;6:355. Scholar
  30. 30.
    Cosentino C, Torres L, Nunez Y, Suarez R, Velez M, Flores M. Hemichorea/Hemiballism associated with hyperglycemia: report of 20 cases. Tremor Other Hyperkinet Mov (N Y). 2016;6:402. Scholar
  31. 31.
    Bejot Y, Giroud M, Moreau T, Benatru I. Clinical spectrum of movement disorders after stroke in childhood and adulthood. Eur Neurol. 2012;68(1):59–64. Scholar
  32. 32.
    Walker RH. Untangling the thorns: advances in the neuroacanthocytosis syndromes. J Mov Disord. 2015;8(2):41–54. Scholar
  33. 33.
    van de Zande NA, Massey TH, McLauchlan D, Pryce Roberts A, Zutt R, Wardle M, et al. Clinical characterization of dystonia in adult patients with Huntington’s disease. Eur J Neurol. 2017;24(9):1140–7. Scholar
  34. 34.
    Termsarasab P, Frucht SJ. The “Stutter-Step”: a peculiar gait feature in advanced Huntington’s disease and chorea-acanthocytosis. Mov Disord Clin Pract. 2018;5(2):223–4. Scholar
  35. 35.
    Termsarasab P, Frucht SJ. Dystonic storm: a practical clinical and video review. J Clin Mov Disord. 2017;4:10. Scholar
  36. 36.
    Dean SL, Singer HS. Treatment of Sydenham’s chorea: a review of the current evidence. Tremor Other Hyperkinet Mov (N Y). 2017;7:456. Scholar
  37. 37.
    Mohammad SS, Nosadini M, Grattan-Smith P, Dale RC. Intravenous immunoglobulin in acute Sydenham’s chorea: a systematic review. J Paediatr Child Health. 2015;51(12):1235–8. Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Division of Movement DisordersNew York University Grossman School of Medicine, The Marlene and Paolo Fresco Institute for Parkinson’s and Movement Disorders, NYU Langone HealthNew YorkUSA
  2. 2.Division of Neurology, Faculty of MedicineRamathibodi Hospital, Mahidol UniversityBangkokThailand

Personalised recommendations