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Chorea

Chorea: Excerpt from In a Page: Signs and Symptoms

Chorea (Greek for “dance”) refers to continuous, rapid, and abrupt jerking movements, which are involuntary and often possess a writhing quality. These movements often interfere with the ability to complete daily activities. A characteristic feature is an inability to maintain voluntary sustained contractions. When chorea is proximal and of large amplitude, it is called ballismus.

Differential Diagnosis

  • Huntington's disease (chronic progressive hereditary chorea)
    –Autosomal dominant transmission
    –Associated with psychiatric symptoms and progressive dementia
    –Caudate atrophy on neuroimaging studies
    –Marker on chromosome 4
    • Sydenham's chorea
      –Symptoms follow febrile illness (20–30%
      of cases are associated with group A strep)
      –Seen in rheumatic fever
      –Peak ages: 5–13 years
      –More common in females
  • Systemic lupus erythematosus
  • AIDS
  • Hyperthyroidism
    • Chorea gravidarum
      –Develops in the first 4–5 months of
      pregnancy
      –Resolves following delivery
  • Drug-induced (e.g., levodopa, stimulants, anticonvulsants, antidepressants, neuroleptics, oral contraceptives)
  • Stroke
  • Neoplasm
    • Wilson's disease
      –Autosomal recessive disorder
      –Deficiency in copper metabolism
      –Associated with hepatic dysfunction,
      dystonia, dysarthria
  • Benign hereditary chorea
    –Autosomal dominant
    –Onset before age 5
    –Symptoms are nonprogressive
    • Neuroacanthocytosis
      –Etiology unknown
      –Characterized by chorea and deformed
      erythrocytes
  • DRPLA
    –Most common in Japan
    –Characterized by chorea, ataxia, epilepsy, and dementia
  • Workup and Diagnosis

    • History and physical examination
      –Clinical diagnosis is sufficient for Sydenham's chorea
      –Huntington's disease may present with psychiatric symptoms (e.g., depression) before other manifestations; onset of symptoms typically occurs in the fourth and fifth decades of life
      –The appearance of Kayser-Fleischer rings in the cornea on slit-lamp exam is diagnostic for Wilson's disease
    • Neuroimaging (CT, MRI) to rule out mass lesions and Huntington's disease (cerebral/basal ganglion atrophy)
    • Genetic testing for Huntington's disease
    • Echocardiography to diagnose carditis
    • Throat culture or serology (ASO) for streptococcal infection
    • Low level of serum ceruloplasmin and elevated 24-hour urine copper in Wilson's disease
    • Thyroid function tests to rule out hyperthyroidism
    • ANA to rule out lupus
    • Neuroacanthocytosis: Acanthocytes appear on peripheral smear with clinical symptoms of chorea, dystonia, and tics
    • DRPLA: Imaging studies may reveal cerebral and cerebellar atrophy

    Treatment

    • Huntington's disease: Antidepressants may reduce depressive symptoms; neuroleptics (e.g., haloperidol, clozapine) may suppress choreic movements; disease is progressive and fatal; genetic counseling is suggested
    • Sydenham's chorea is usually self-limited with symptom resolution within 15 weeks
    • Acute rheumatic fever: Corticosteroids may shorten course of chorea; antibiotic therapy with penicillin for at least 10 days
    • Drug-induced chorea: Discontinue or reduce dosage of implicated medications; atypical neuroleptics are associated with decreased risk of involuntary movements
    • Chorea gravidarum may require delivery
    • Wilson's disease: Copper-chelating agents
    • Neuroacanthocytosis: Usually fatal within 9 years of symptom onset
    • Specific therapy for lupus, hyperthyroidism, AIDS

    Book Source Details

    • Book Title: In a Page: Signs and Symptoms
    • Author(s): Scott Kahan, Ellen G. Smith
    • Year of Publication: 2004
    • Copyright Details: In a Page: Signs and Symptoms, Copyright © 2004 Lippincott Williams & Wilkins.

    More About Sydenham chorea

    More Medical Textbooks Online about Sydenham chorea

    Review other book chapters online related to Sydenham chorea:

    Medical Books Excerpts
    • Chorea
    • "In a Page: Signs and Symptoms" (2004)
    • Chorea
    • "In A Page: Pediatric Signs and Symptoms" (2007)
    • CHOREA
    • "Differential Diagnosis in Primary Care" (2007)
    • Chorea
    • "Signs & Symptoms: A 2-in-1 Reference for Nurses" (2007)
    • CHOREA
    • "Differential Diagnosis in Primary Care" (2007)
     

    Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.




    More About This Book:
    Title: In a Page: Signs and Symptoms
    Authors: Scott Kahan, Ellen G. Smith
    Publisher: Lippincott Williams & Wilkins
    Copyright: 2004
    ISBN: 1-4051-0368-X

     » Next page: Chorea (In A Page: Pediatric Signs and Symptoms)

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