Chorea
| Chorea | |
| ICD-10 | G25.5 |
|---|---|
| ICD-9 | 333.5 |
| DiseasesDB | 16662 |
| MeSH | D002819 |
Template:Search infobox Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Chorea sancti viti (Latin for "St. Vitus' dance") is an abnormal involuntary movement disorder, one of a group of neurological disorders called dyskinesias. The term chorea is derived from a Greek word χορεία (a kind of dance), as the quick movements of the feet or hands are vaguely comparable to dancing or piano playing.
Presentation
Chorea is characterized by brief, irregular contractions that are not repetitive or rhythmic, but appear to flow from one muscle to the next.
These 'dance-like' movements of chorea (from the same root word as "choreography") often occur with athetosis, which adds twisting and writhing movements.
Causes
Chorea can occur in a variety of conditions and disorders.
- Chorea is a primary feature of Huntington's disease, a progressive, hereditary movement disorder.
- Twenty percent of children and adolescents with rheumatic fever develop Sydenham's chorea as a complication.
- Chorea may also be caused by drugs (levodopa, anti-convulsants, anti-psychotics), metabolic disorders, endocrine disorders, and vascular incidents.
Causes by Organ System
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| Cardiovascular | No underlying causes |
| Chemical / poisoning | |
| Dermatologic | No underlying causes |
| Drug Side Effect | |
| Ear Nose Throat | No underlying causes |
| Endocrine | |
| Environmental | No underlying causes |
| Gastroenterologic | No underlying causes |
| Genetic | |
| Hematologic | |
| Iatrogenic | No underlying causes |
| Infectious Disease | |
| Musculoskeletal / Ortho | No underlying causes |
| Neurologic |
Olivopontocerebellar atrophy 1 Idiopathic basal ganglia calcification Dentatorubropallidoluysian degeneration Neuhauser- Eichner –Opitz syndrome Alternating hemiplegia of childhood Spinocerebeller ataxia recessive 1 Microcephaly-pontocerebellarhypoplasia-dyskinesia Cross- McKusick-Breen syndrome Paroxysmal kinesigenic choreoathetosis Paroxysmal nonkinesigenic choreathetosis |
| Nutritional / Metabolic |
Pyruvate decarboxylase deficiency 3- Methylglutaconic aciduria type 3 |
| Obstetric/Gynecologic | |
| Oncologic | No underlying causes |
| Opthalmologic | No underlying causes |
| Overdose / Toxicity | No underlying causes |
| Psychiatric | No underlying causes |
| Pulmonary | No underlying causes |
| Renal / Electrolyte | |
| Rheum / Immune / Allergy | |
| Sexual | No underlying causes |
| Trauma | No underlying causes |
| Urologic | No underlying causes |
| Miscellaneous |
Ballism
When chorea is serious, slight movements will become thrashing motions; this form of severe chorea is referred to as ballism. Walking may become peculiar, and include odd postures and leg movements. Unlike ataxia and dystonia, which affect the quality of voluntary movements or parkinsonism, which is a hindrance of voluntary movements, the movements of chorea and ballism occur on their own, without conscious effort.
Causes In alphabetical order. [1] [2]
Olivopontocerebellar atrophy 1
Idiopathic basal ganglia calcification
Dentatorubropallidoluysian degeneration
Neuhauser- Eichner –Opitz syndrome
Alternating hemiplegia of childhood
Spinocerebeller ataxia recessive 1
Microcephaly-pontocerebellarhypoplasia-dyskinesia
Cross- McKusick-Breen syndrome
Paroxysmal kinesigenic choreoathetosis
Paroxysmal nonkinesigenic choreathetosis
Gilles de la tourette syndrome
Pyruvate decarboxylase deficiency
3- Methylglutaconic aciduria type 3
3- methyglutaconic aciduria type 3
Infantile epileptic – dyskinetic encephalopathy
Diagnosis
History and Symptoms
- Complete history required
- Psychiatric symptoms (Huntington's)
Appearance of the Patient
Eyes
- Appearance of Kayser-Fleischer rings in cornea is diagnostic for Wilson's disease (slit-lamp examination)
Laboratory Findings
- Antinuclear antibody (ANA) to diagnose lupus
- Serology (ASO) or throat cultures required to rule out streptococcal infection
- Urinalysis (Wilson's)
- Serum ceruloplasmin (Wilson's)
- Thyroid function tests to diagnose hyperthyroidism
MRI and CT
- CT scans and/or MRIs are required to rule out Huntington's disease and mass lesions
Echocardiography or Ultrasound
- In order to diagnose carditis, an ECG may be indicated
Other Imaging Findings
- In order to reveal cerebral and cerebellar atrophies in patients with DRPLA, various imaging studies are indicated
Other Diagnostic Studies
- Genetic testing may be required for those patients suspected of having Huntington's disease
- Peripheral smear for diagnostic neuroacanthocytosis
Treatment
- Disease-specific treatment for AIDS, hyperthyroidism, lupus
- Symptom resolution occurs within 15 weeks for those patients with Sydenham's chorea
- Within nine years of onset of symptoms, neuroacanthocytosis is usually fatal
- Discontinue use of drugs that may have caused drug-induced chorea
- Genetic counseling is usually recommended for those patients with Huntington's disease
| Cause | Treatment |
|---|---|
| Huntington's disease | A common treatment is dopaminergic antagonists, although treatment is largely supportive. |
| Sydenham's chorea | Usually involves antibiotic drugs to treat the infection, followed by drug therapy to prevent recurrence. |
| Drug-induced chorea. | Adjusting medication dosages. |
| Metabolic and endocrine-related choreas | Treated according to the cause(s) of symptoms. |
Acute Pharmacotherapies
- For patients with Wilson's disease, copper-chelating agents are recommended
- For patients with acute rheumatic fever, antibiotic therapy and possibly corticosteroids
- For patients with Huntington's disease, antidepressants, neuroleptics
References
See also
Template:Diseases of the nervous system