Chorea

Nawaz K.A. Hack, M.D.
Joseph S. Kass, M.D., J.D.

Basic Information

Definition

Chorea is the term used to describe brief movements that involve contiguous muscle groups. It comes from the Greek word meaning dance.

Synonyms

Sydenham’s chorea
Saint Vitus dance

ICD-10CM CODES
G25.4	Drug-induced chorea
G25.5	Other chorea
I02.0	Rheumatic chorea with heart involvement
I02.9	Rheumatic chorea without heart involvement

Epidemiology & Demographics

Incidence

No published data exist on the incidence of chorea in the United States.

Predominant Sex and Age

Can affect any age and sex depending on the cause

Risk Factors

The most important risk factors are a family history of Huntington’s disease, exposure to rheumatic fever as a child, or being pregnant with a history of rheumatic fever.

Physical Findings & Clinical Presentation

Presentation depends on the underlying cause. Huntington’s disease typically presents with neuropsychiatric symptoms predating choreiform movements. In the case of Sydenham’s chorea, drug-induced chorea, or chorea gravidarum, hyperkinetic movements involving the hands and face may be noticed first. Nonrhythmic, brief, contiguous movement and facial grimacing can be clues to the presence of chorea. Writhing movement of the hands or feet is known as athetosis. An enlarged tongue and self-mutilation may accompany chorea in patients with neuroacanthocytosis. Choreiform movements tend to be suppressed during physical examination; look for suppressive techniques such as holding of the face or crossing of the legs.

Etiology

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Genetic causes include Huntington’s disease, neuroacanthocytosis, various spinocerebellar ataxias, such as SCA3, dentatorubral-pallidoluysian atrophy (DRPLA), neurodegeneration with brain iron accumulation, and Wilson’s disease.
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Infectious causes include rheumatic fever causing Sydenham’s chorea (most common cause of chorea in children), chorea gravidarum during pregnancy, and prion disease.
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Drug-induced causes include oral contraceptives, tricyclic antidepressants, cimetidine, digoxin, verapamil, baclofen, steroids, antiepileptics, and neuroleptics. Tardive dyskinesia, most typically from dopamine-blocking agents such as neuroleptics or some antiemetics, can result in choreiform movements.
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Hemichorea may be due to antiphospholipid antibody syndrome, systemic lupus erythematosus, or hemichorea-hemiballismus syndrome due to severe hyperglycemia or stroke, typically in the thalamus or subthalamic nucleus.
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Infectious causes of chorea include prion disease and cryptococcal meningitis in HIV patients.

Diagnosis

Differential Diagnosis

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Huntington’s disease
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Tardive dyskinesia
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Dystonia
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Tic disorders
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Stroke
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Prion disease
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Rheumatic fever
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Conversion disorder

Laboratory Tests

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Complete metabolic panel, including liver function tests
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CBC with peripheral smear looking for acanthocytes
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Serum copper and ceruloplasmin levels
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Thyroid function tests
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Antistreptolysin O antibodies
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Genetic testing for Huntington’s disease should be done at a comprehensive center involving neurology, psychology, and genetics; complete team support is required before genetic testing is undertaken.

Imaging Studies

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MRI of the brain with and without contrast.
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Echocardiogram if rheumatic fever is suspected.

Treatment

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Depending on the cause, treatment must be tailored.
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Chorea related to Huntington’s disease can be treated with tetrabenazine or deutetrabenazine (recently approved by the FDA). Caution must be exercised because the medication puts the patient at significant risk for depression.
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Withdrawing the offending drug can treat drug-induced chorea.
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Some conditions such as SCA3 or neuroacanthocytosis and Huntington’s disease have a strong neuropsychiatric component and may need to be comanaged with behavioral health specialists.

Nonpharmacologic Therapy

Rehabilitative services such as physical therapy, occupational therapy, and speech therapy are important for genetic forms of chorea.

Cognitive therapy is now becoming more important in maintaining quality of life in most of these disorders.

Acute General Rx

Sudden-onset chorea warrants brain imaging to evaluate for stroke.

MRI of the brain is important for full evaluation of chorea, as it allows for the evaluation of basal ganglia structure. Huntington’s disease is associated with atrophy of the head of the caudate.

Chronic Rx

Tetrabenazine and deutetrabenazine are the only medications currently approved by the FDA to treat the chorea of Huntington’s disease. These medications reduce the chorea but do not halt the underlying neurodegenerative process of Huntington’s disease.

Deutetrabenazine and valbenazine are the only medications currently approved by the FDA for treating tardive dyskinesia.

A multidisciplinary approach is useful for treating Huntington’s disease.

Complementary Medicine

There are case reports showing some efficacy in acupuncture for the treatment of temporomandibular chorea in a patient with Sydenham’s chorea.

Disposition

Sydenham’s chorea and chorea gravidarum tend to resolve in a few weeks.

Huntington’s disease is a progressive neurodegenerative disorder.

Tardive dyskinesia due to neuroleptics may be treated medically, and offending agents should be withdrawn, but comanagement with a psychiatrist is imperative to avoid destabilization of the patient and to monitor for depression using TD-specific therapy.

Referral

Refer to a neurologist.

Pearls & Considerations

Comments

Chorea is a symptom of an underlying disorder. Investigations are targeted to identify reversible causes.

Prevention

Treat streptococcal throat infection early to prevent future complications.

Weight risk-benefit in using medications known to cause tardive dyskinesia

Patient/Family Education

For Huntington’s disease and other genetic forms of chorea, it is important to provide support and refer to support groups.

Ferri – Chorea