Chorea

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

• Chorea gravidarum
  –Develops in the first 4–5 months of
  pregnancy
  –Resolves following delivery

• Wilson's disease
  –Autosomal recessive disorder
  –Deficiency in copper metabolism
  –Associated with hepatic dysfunction,
  dystonia, dysarthria

• Neuroacanthocytosis
  –Etiology unknown
  –Characterized by chorea and deformed
  erythrocytes

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.

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Copyright Details: In a Page: Signs and Symptoms, Copyright © 2008 Williams & Wilkins.

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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

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