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

Cardiovascular	No underlying causes
Chemical / poisoning	Carbon monoxide toxicity , Toulene poisoning
Dermatologic	No underlying causes
Drug Side Effect	Amphetamines , Ethotoin , Lamotrigine , Levodopa , Dopamine agonists , Levomepromazine , Lithium , Methadone , Oral contraceptive pills , Phenothiazines , Phenytoin , Tolcapone , Tricyclic antidepressants
Ear Nose Throat	No underlying causes
Endocrine	Hypoglycaemia , Hypoparathyroidism, Thyrotoxicosis .
Environmental	No underlying causes
Gastroenterologic	No underlying causes
Genetic	De Barsy syndrome , McLeod phenotype
Hematologic	Ceruloplasmin deficiency , Polycythemia , Porphyria , Henoch-Schönlein purpura
Iatrogenic	No underlying causes
Infectious Disease	Rheumatic Fever , HIV
Musculoskeletal / Ortho	No underlying causes
Neurologic	Huntington’s disease , Movement disorders , Ganglion , Wilson’s disease , Neuroacanthocytosis , Paroxysmal choreoathetosis , Cerebral birth injury , Kernicterus , cerebral trauma , Lacunar infarction , Creutzfeldt- Jacob disease , Cebral Palsy Spinocerebellar ataxia 17 Olivopontocerebellar atrophy 1 Idiopathic basal ganglia calcification , Hallervorden – Spatz disease , Troyer syndrome , Dentatorubropallidoluysian degeneration , Westphal disease , Neuhauser- Eichner –Opitz syndrome , Alternating hemiplegia of childhood , Spinocerebeller ataxia recessive 1 , Microcephaly-pontocerebellarhypoplasia-dyskinesia , Ataxia – telangiectasia , Cross- McKusick-Breen syndrome , Paroxysmal kinesigenic choreoathetosis , Neuroferritinopathy , Wolfram’s disease , Mount Reback syndrome , Paroxysmal nonkinesigenic choreathetosis , Gilles de la tourette syndrome , Fahr's syndrome , Familial benign chorea.
Nutritional / Metabolic	Pyruvate decarboxylase deficiency , 3- Methylglutaconic aciduria type 3 , Benedikt’s syndrome , Infantile epileptic – dyskinetic encephalopathy , Ceroid lipofuscinosis , Glutaric aciduria type 1 , Lesch-Nyhan syndrome
Obstetric/Gynecologic	Pregnancy
Oncologic	No underlying causes
Opthalmologic	No underlying causes
Overdose / Toxicity	No underlying causes
Psychiatric	No underlying causes
Pulmonary	No underlying causes
Renal / Electrolyte	Henosch - scholein purpura
Rheum / Immune / Allergy	SLE , Henosch - scholein purpura
Sexual	No underlying causes
Trauma	No underlying causes
Urologic	No underlying causes
Miscellaneous	Arterio-venous malformations , Senile chorea , Haw river syndrome

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.

• Amphetamines

• Carbon monoxide toxicity

• Ceruloplasmin deficiency

• De Barsy syndrome

• Ethotoin

• Hypoglycaemia

• Hypoparathyroidism.

• Lamotrigine

• Levodopa

• Dopamine agonists

• Levomepromazine

• Lithium

• McLeod phenotype

• Methadone

• Oral contraceptive pills

• Phenothiazines

• Phenytoin

• Polycythemia

• Porphyria

• Thyrotoxicosis

• Tolcapone

• Toulene poisioning

• Tricyclic antidepressants

• Rheumatic Fever

• HIV

• Huntington’s disease

• Movement disorders

• Ganglion

• Wilson’s disease

• Neuroacanthocytosis

• Paroxysmal choreoathetosis

• Cerebral birth injury

• Kernicterus

• cerebral trauma

• Lacunar infarction

• Creutzfeldt- Jacob disease

• Cebral Palsy

• Spinocerebellar ataxia 17

• Olivopontocerebellar atrophy 1

• Idiopathic basal ganglia calcification

• Hallervorden – Spatz disease

• Troyer syndrome

• Dentatorubropallidoluysian degeneration

• Westphal disease

• Neuhauser- Eichner –Opitz syndrome

• Alternating hemiplegia of childhood

• Spinocerebeller ataxia recessive 1

• Microcephaly-pontocerebellarhypoplasia-dyskinesia

• Ataxia – telangiectasia

• Cross- McKusick-Breen syndrome

• Paroxysmal kinesigenic choreoathetosis

• Neuroferritinopathy

• Wolfram’s disease

• Mount Reback syndrome

• Paroxysmal nonkinesigenic choreathetosis

• Gilles de la tourette syndrome

• Fahr’s disease

• Familial benign chorea

• Pyruvate decarboxylase deficiency

• 3- Methylglutaconic aciduria type 3

• Benedikt’s syndrome

• Infantile epileptic – dyskinetic encephalopathy

• Ceroid lipofuscinosis

• Glutaric aciduria type 1

• Lesch-Nyhan syndrome

• Pregnancy

• Respiratory failure

• SLE

• Henoch-Schönlein purpura

• Arteriovenous malformations

• Senile chorea

• Haw river syndrome

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

See also

• Movement disorder
• Choreoathetosis