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
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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
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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
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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)
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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
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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)
- [ read ]
- Chorea
- "In A Page: Pediatric Signs and Symptoms" (2007)
- [ read ]
- CHOREA
- "Differential Diagnosis in Primary Care" (2007)
- [ read ]
- Chorea
- "Signs & Symptoms: A 2-in-1 Reference for Nurses" (2007)
- [ read ]
- CHOREA
- "Differential Diagnosis in Primary Care" (2007)
- [ read ]
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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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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» Next page: Chorea (In A Page: Pediatric Signs and Symptoms)
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