Skip to main content
SpringerLink
Log in

Chorea

  • Chapter
  • First Online:
Book cover Differential Diagnosis of Movement Disorders in Clinical Practice

  • 1768 Accesses

Abstract

Chorea is characterized by involuntary, brief, unpredictable, and random hyperkinetic movements. This chapter reviews main clinical characteristics of chorea and similar hyperkinetic movements and their most common causes. We describe characteristic phenotypic features of chorea-causing conditions, their differential diagnosis, and most useful clinical work-up, including genetic testing. We also discuss most typical clinical presentation of the most common types of chorea, especially Huntington’s disease with its motor and non-motor symptoms and signs, and main therapeutic options. Additional emphasis is on treatable causes of chorea, such as Sydenham chorea and Wilson’s disease.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 39.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight 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

Bibliography

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

    Article  PubMed  Google Scholar 

  • Bader B, Vollmar C, Ackl N, Ebert A, la Fougère C, Noachtar S, Danek A. Bilateral temporal lobe epilepsy confirmed with intracranial EEG in chorea-acanthocytosis. Seizure. 2011;20:340–2.

    Article  PubMed  Google Scholar 

  • Baizabal-Carvallo JF, Alonso-Juarez M, Koslowski M. Chorea in systemic lupus erythematosus. J Clin Rheumatol. 2011;17:69–72.

    Article  PubMed  Google Scholar 

  • Breedveld GJ, Percy AK, MacDonald ME, et al. Clinical and genetic heterogeneity in benign hereditary chorea. Neurology. 2002;59:579–84.

    Article  PubMed  CAS  Google Scholar 

  • Brilot F, Merheb V, Ding A, Murphy T, Dale RC. Antibody binding to neuronal surface in Sydenham chorea, but not in PANDAS or Tourette syndrome. Neurology. 2011;76:1508–13.

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  • Cardoso F. Sydenham’s chorea. Handb Clin Neurol. 2011;100:221–9.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  • Carroll E, Sanchez-Ramos J. Hyperkinetic movement disorders associated with HIV and other viral infections. Handb Clin Neurol. 2011;100:323–34.

    Article  PubMed  Google Scholar 

  • Cortese I, Chaudhry V, So YT, Cantor F, Cornblath DR, Rae-Grant A. Evidence-based guideline update: plasmapheresis in neurologic disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2011;76:294–300.

    Article  PubMed  CAS  Google Scholar 

  • Cummins A, Eggert J, Pruitt R, Collins JS. Huntington disease: implications for practice. Nurse Pract. 2011;36:41–7.

    Article  PubMed  Google Scholar 

  • Dale RC, Yin K, Ding A, et al. Antibody binding to neuronal surface in movement disorders associated with lupus and antiphospholipid antibodies. Dev Med Child Neurol. 2011;53:522–8.

    Article  PubMed  Google Scholar 

  • de Tommaso M, Serpino C, Sciruicchio V. Management of Huntington’s disease: role of tetrabenazine. Ther Clin Risk Manag. 2011;7:123–9.

    Article  PubMed  Google Scholar 

  • Docherty MJ, Burn DJ. Hyperthyroid chorea. Handb Clin Neurol. 2011;100:279–86.

    Article  PubMed  Google Scholar 

  • Eidelberg D, Surmeier DJ. Brain networks in Huntington disease. J Clin Invest. 2011;121:484–92.

    Article  PubMed  CAS  Google Scholar 

  • Em JJ, Chang MK. Hemiballism-hemichorea and non-ketotic hyperglycaemia. J Neurol Neurosurg Psychiatry. 1994;57:748–50.

    Article  Google Scholar 

  • Goetz CG, Pappert EJ. Textbook of clinical neurology. 2nd ed. Philadelphia: Saunders; 1999.

    Google Scholar 

  • Hagiwara K, Tominaga K, Okada Y, et al. Post-streptococcal chorea in an adult with bilateral striatal encephalitis. J Clin Neurosci. 2011;18:708–9.

    Article  PubMed  Google Scholar 

  • Ikeuchi T, Koide R, Onodera O, et al. Dentatorubral – pallidoluysian atrophy (DRPLA): molecular basis for wide clinical features of DRPLA. Clin Neurosci. 1995;3:23–7.

    PubMed  CAS  Google Scholar 

  • Illarioshkin SN, Igarashi S, Onodera O, et al. Trinucleotide repeat length and rate of progression of Huntington’s disease. Ann Neurol. 1994;36:630–5.

    Article  PubMed  CAS  Google Scholar 

  • Inzelberg R, Weinberger M, Gak E. Benign hereditary chorea: an update. Parkinsonism Relat Disord. 2011;17:301–7.

    Article  PubMed  Google Scholar 

  • Ishaq S, Khalil S, Khan A, Khalid U. Chorea as an unusual presenting feature of anti-phospholipid syndrome. J Pak Med Assoc. 2010;60:975–6.

    PubMed  Google Scholar 

  • Jankovic J, Tolosa E. Parkinson’s disease and movement disorder. 5th ed. Philadelphia: Lippincott Williams and Wilkins; 2007.

    Google Scholar 

  • Johri A, Chaturvedi RK, Beal MF. Hugging tight in Huntington’s. Nat Med. 2011;17:245–6.

    Article  PubMed  CAS  Google Scholar 

  • Kimber TE, Thompson PD. Senile chorea. Handb Clin Neurol. 2011;100:213–7.

    Article  PubMed  CAS  Google Scholar 

  • Kleiner-Fisman G. Benign hereditary chorea. Handb Clin Neurol. 2011;100:199–212.

    Article  PubMed  Google Scholar 

  • Klempíř J, Zidovská J, Stochl J, Ing VK, Uhrová T, Roth J. The number of CAG repeats within the normal allele does not influence the age of onset in Huntington’s disease. Mov Disord. 2011;26:125–9.

    Article  PubMed  Google Scholar 

  • Kobayashi K, Aoyama N, Sasaki J, et al. MRI appearance of a cerebral cavernous malformation in the caudate nucleus before and after chorea onset. J Clin Neurosci. 2011;18:719–21.

    Article  PubMed  Google Scholar 

  • Kremer HP. Imaging Huntington’s disease (HD) brains – imagine HD trails. Neurol Neurosurg Psychiatry. 2005;76:620.

    Article  CAS  Google Scholar 

  • Kremer B, Goldberg P, Andrew SE, et al. A worldwide study of the Huntington’s disease mutation. The sensitivity and specificity of measuring CAG repeats. N Engl J Med. 1994;330:1401–6.

    Article  PubMed  CAS  Google Scholar 

  • Kuehn BM. Imaging helps to identify early changes associated with Huntington disease. JAMA. 2011;305:138.

    Article  PubMed  CAS  Google Scholar 

  • Le Ber I, Camuzat A, Castelnova G, et al. Prevalence of dentatorubral pallidoluysian atrophy in a large series of white patients with cerebellar ataxia. Arch Neurol. 2003;60:1097–9.

    Article  PubMed  Google Scholar 

  • Leung JG, Breden EL. Tetrabenazine for the treatment of tardive dyskinesia. Ann Pharmacother. 2011;45:525–31.

    Article  PubMed  CAS  Google Scholar 

  • Lewin AB, Storch EA, Murphy TK. Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus in identical siblings. J Child Adolesc Psychopharmacol. 2011;21:177–82.

    Article  PubMed  Google Scholar 

  • Marder K, Zhao H, Myers RH, et al. Rate of functional decline in Huntington’s disease. Neurology. 2000;54:452–8.

    Article  PubMed  CAS  Google Scholar 

  • Margolis RL, Homes SE, Rosenblatt A, et al. Huntington’s disease like 2 (HDL2) in North America and Japan. Ann Neurol. 2004;56:670–4.

    Article  PubMed  CAS  Google Scholar 

  • Marshall FJ. A randomized, double blind placebo-controlled study of tetrabenazine in patients with Huntington’s disease. Mov Disord. 2004;19:1122.

    Google Scholar 

  • Marvi MM, Lew MF. Polycythemia and chorea. Handb Clin Neurol. 2011;100:271–6.

    Article  PubMed  Google Scholar 

  • Miyasaki JM. Chorea caused by toxins. Handb Clin Neurol. 2011;100:335–46.

    Article  PubMed  Google Scholar 

  • Mochel F, Haller RG. Energy deficit in Huntington disease: why it matters. J Clin Invest. 2011;121:493–9.

    Article  PubMed  CAS  Google Scholar 

  • Ondo WG. Hyperglycemic nonketotic states and other metabolic imbalances. Handb Clin Neurol. 2011;100:287–91.

    Article  PubMed  Google Scholar 

  • Paulsen JS, Ready RE, Hamilton JM, Mega MS, Cummings JL. Neuropsychiatric aspects of Huntington’s disease. J Neurol Neurosurg Psychiatry. 2001;71:310–4.

    Article  PubMed  CAS  Google Scholar 

  • Paulsen JS, Hoth KF, Nehl C, Stierman L. Critical periods of suicide risk in Huntington’s disease. Am J Psychiatry. 2005;162:725–31.

    Article  PubMed  Google Scholar 

  • Perlman SL. Spinocerebellar degenerations. Handb Clin Neurol. 2011;100:113–40.

    Article  PubMed  Google Scholar 

  • 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 

  • Przekop A, Sanger TD. Birth-related syndromes of athetosis and kernicterus. Handb Clin Neurol. 2011;100:387–95.

    Article  PubMed  Google Scholar 

  • Przekop A, McClure C, Ashwal S. Postoperative encephalopathy with choreoathetosis. Handb Clin Neurol. 2011;100:295–305.

    Article  PubMed  Google Scholar 

  • Ray LW, Koller WC. Movement disorders, neurologic principles and practice. 2nd ed. New York: McGraw-Hill; 1997.

    Google Scholar 

  • Reglodi D, Kiss P, Lubics A, Tamas A. Review on the protective effects of PACAP in models of neurodegenerative diseases in vitro and in vivo. Curr Pharm Des. 2011;17:962–72.

    Article  PubMed  CAS  Google Scholar 

  • Robottom BJ, Weiner WJ. Chorea gravidarum. Handb Clin Neurol. 2011;100:231–5.

    Article  PubMed  Google Scholar 

  • Rolands LP, editor. Merritt’s textbook of neurology. 10th ed. New York: Lippincott Williams & Wilkins; 2000.

    Google Scholar 

  • Rozas JL, Gómez-Sánchez L, Tomás-Zapico C, Lucas JJ, Fernández-Chacón R. Increased neurotransmitter release at the neuromuscular junction in a mouse model of polyglutamine disease. J Neurosci. 2011;31:1106–13.

    Article  PubMed  CAS  Google Scholar 

  • Sadeghian H, O'Suilleabhain PE, Battiste J, Elliott JL, Trivedi JR. Huntington chorea presenting with motor neuron disease. Arch Neurol. 2011;68:650–2.

    Article  PubMed  Google Scholar 

  • Sah DW, Aronin N. Oligonucleotide therapeutic approaches for Huntington disease. J Clin Invest. 2011;121:500–7.

    Article  PubMed  CAS  Google Scholar 

  • Schneider SA, Bhatia KP. Huntington’s disease look-alikes. Handb Clin Neurol. 2011;100:101–12.

    Article  PubMed  Google Scholar 

  • Shirendeb U, Reddy AP, Manczak M, et al. Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington’s disease: implications for selective neuronal damage. Hum Mol Genet. 2011;20:1438–55.

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  • Stevanin G, Fujigasaki H, Lebre AS, et al. Huntington’s disease like phenotype due to trinucleotide repeat expansions in the TBP and JPH3 genes. Brain. 2003;126:1599–603.

    Article  PubMed  Google Scholar 

  • 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 

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

    Article  PubMed  CAS  Google Scholar 

  • Walker HK. An overview of the nervous system. In: Walker HK, Hall WD, Hurst JW, editors. Clinical methods: the history, physical, and laboratory examinations. 3rd ed. Boston: Butterworths; 1990a. Chapter 50.

    Google Scholar 

  • Walker HK. Involuntary movements. In: Walker HK, Hall WD, Hurst JW, editors. Clinical methods: the history, physical, and laboratory examinations. 3rd ed. Boston: Butterworths; 1990b. Chapter 70.

    Google Scholar 

  • Walker RH, Rasmussen A, Rudnicki D, et al. Huntington’s disease like 2 can present as chorea acanthocytosis. Neurology. 2003;61:1002–4.

    Article  PubMed  CAS  Google Scholar 

  • Walker RH, Jung HH, Danek A. Neuroacanthocytosis. Handb Clin Neurol. 2011;100:141–51.

    Article  PubMed  Google Scholar 

  • Walterfang M, Evans A, Looi JC, et al. The neuropsychiatry of neuroacanthocytosis syndromes. Neurosci Biobehav Rev. 2011a;35:1275–83.

    Article  PubMed  Google Scholar 

  • Walterfang M, Looi JC, Styner M, et al. Shape alterations in the striatum in chorea-acanthocytosis. Psychiatry Res. 2011b;192:29–36.

    Article  PubMed  Google Scholar 

  • Zesiewicz TA, Sullivan KL. Drug-induced hyperkinetic movement disorders by nonneuroleptic agents. Handb Clin Neurol. 2011;100:347–63.

    Article  PubMed  Google Scholar 

  • Zijlmans JC. Vascular chorea in adults and children. Handb Clin Neurol. 2011;100:261–70.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Parkinson’s Clinic of Eastern Toronto, Toronto, ON, Canada

    Abdul Qayyum Rana

  2. Department of Neurology Division of Movement Disorders, Vanderbilt University, Nashville, TN, USA

    Peter Hedera

Authors
  1. Abdul Qayyum Rana
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Peter Hedera
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Rana, A.Q., Hedera, P. (2014). Chorea. In: Differential Diagnosis of Movement Disorders in Clinical Practice. Springer, Cham. https://doi.org/10.1007/978-3-319-01607-8_3

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-01607-8_3

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-01606-1

  • Online ISBN: 978-3-319-01607-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation