Chorea

Chorea: Excerpt from In A Page: Pediatric Signs and Symptoms

Chorea consists of brief, irregular, nonrhythmic, unsustained involuntary movements that flow from one part of the body to another. Chorea is often accompanied by athetosis, which are slow, writhing, involuntary movements. These movement disorders are thought to result from dysfunction of the basal ganglia.

Differential Diagnosis

• Toxins
  –Neuroleptics, phenytoin, antiemetics, oral contraceptives, theophylline, L-dopa, stimulants, lithium, carbon monoxide, manganese
• Sydenham chorea (in rheumatic fever)
  –Migratory chorea, hypotonia, dysarthria, emotional liability
  –Usually 4 months after group A β-hemolytic Streptococcus infection
  –Molecular mimicry between streptococcal and CNS antigens results in formation of cross-reactive antibodies that disrupt basal ganglia function
  –Carditis is present in 80% of Sydenham chorea patients
  • Inherited choreas
    –Benign familial chorea
    –Juvenile Huntington chorea (usually presents with rigidity)
    –Familial paroxysmal choreoathetosis
  • Postinfectious: Mycoplasma, HSV, EBV, echovirus 25, varicella
  • Encephalitis: viral, mycoplasma, Lyme
  • Post-cardiac surgery
    –“Post-pump chorea”
    –Usually 2 weeks after cardiac surgery
  • Syndrome or disease associated
    –Wilson disease
    –Hallervorden-Spatz (disorder of iron metabolism with degeneration of globus pallidus)
    –Fahr disease: Encephalopathy and progressive calcification of basal ganglia
    –Lesch-Nyhan syndrome
    –Ataxia-telangiectasia
  • Endocrine: Hyperthyroidism, pregnancy (chorea gravidarum)
  • Acquired brain disorders
    –Multiple sclerosis, basal ganglia stroke, hypoxic ischemic encephalopathy, neoplasm
  • Abetalipoproteinemia
  • Glutaric aciduria type I
  • Neuroacanthocytosis
  • Systemic lupus erythematosus
  • Kernicterus
  • Antiphospholipid antibody syndrome
  • Mitochondrial encephalopathies

  Workup and Diagnosis

  • History
    –Fever, rash associated symptoms
    –History of streptococcal infections, rheumatic fever, arthritis
    –Birth history, family history, medications, ingestions
  • Physical exam
    –Eye exam: Kayser-Fleischer rings (Wilson disease)
    –Cardiac, joint, and skin exam (for rheumatic fever)
    –Neurologic exam should include eye movement, dysarthria, evaluation of tone, motor impersistence
  • Labs
    –Electrolytes, glucose, calcium, magnesium, LFTs, BUN/Cr, acetate, pyruvate
    –TSH, parathyroid hormone, hCG
    –CBC and blood smear (for acanthocytes)
    –Throat culture, antistreptolysin O titer
    –Ceruloplasmin (low in Wilson disease), amino acids
    –Lyme titer
    –Anti-nuclear, anti-cardiolipin, anti-phospholipid, anti dsDNA antibodies
    –Rheumatoid factor, lipid profile
    –Huntington disease genetic testing
    • Studies
      –Cardiac evaluation with Echo, ECG
      –LP useful in infectious or postinfectious cases
            –Multiple sclerosis (find oligoclonal bands)
      –Neuroimaging: CT or preferably MRI

    Treatment

    • Treatment should be reserved for patients in whom chorea severity interferes with function
      –Simple measures such as rest and avoidance of stress often alleviate symptoms
      –Anti-epilepsy medications are used for paroxysmal choreas
      –Dopamine blockers such as haloperidol and pimozide are used to decrease the movement disorder
    • Sydenham chorea
      –May be treated with valproate or pimozide
      –Immunosuppression with steroids, intravenous immunoglobulin or plasmapheresis have been used
      –Secondary prophylaxis with penicillin is required
    • Wilson disease: Treat with penicillamine, trientene chelation, or zinc
    • In cases of toxin-induced chorea, removal of the offending agent is usually sufficient

Book Source Details

• Book Title: In A Page: Pediatric Signs and Symptoms
• Author(s): Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan
• Year of Publication: 2007
• Copyright Details: In A Page: Pediatric Signs and Symptoms, Copyright © 2007 Lippincott Williams & Wilkins.

More About Dentatorubral Pallidoluysian Atrophy

• Back to disease: Dentatorubral Pallidoluysian Atrophy: Introduction
• Next Book Extract About Dentatorubral Pallidoluysian Atrophy: Seizures – Childhood (In A Page: Pediatric Signs and Symptoms)
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: In A Page: Pediatric Signs and Symptoms Authors: Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan Publisher: Lippincott Williams & Wilkins Copyright: 2007 ISBN: 1-4051-0427-9

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• Seizures – Childhood (In A Page: Pediatric Signs and Symptoms)
• Seizures – Neonatal (In A Page: Pediatric Signs and Symptoms)
• CHOREA (Differential Diagnosis in Primary Care)
• FASCICULATIONS (Differential Diagnosis in Primary Care)
• MYOCLONUS (Differential Diagnosis in Primary Care)

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