Chorea (disease)
Encyclopedia
Choreia is an abnormal involuntary movement disorder
Movement disorder
Movement disorders include:* Akathisia * Akinesia * Associated Movements * Athetosis...

, one of a group of neurological disorders called dyskinesia
Dyskinesia
Dyskinesia is a movement disorder which consists of effects including diminished voluntary movements and the presence of involuntary movements, similar to tics or choreia. Dyskinesia can be anything from a slight tremor of the hands to uncontrollable movement of, most commonly, the upper body but...

s. The term choreia is derived from the Greek word χορεία (=dance), see choreia (dance), as the quick movements of the feet or hands are vaguely comparable to dancing or piano
Piano
The piano is a musical instrument played by means of a keyboard. It is one of the most popular instruments in the world. Widely used in classical and jazz music for solo performances, ensemble use, chamber music and accompaniment, the piano is also very popular as an aid to composing and rehearsal...

 playing.

The term hemichoreia refers to choreia of one side of the body, such as choreia of one arm and not both (comparable to hemiballismus
Hemiballismus
Hemiballismus is a very rare movement disorder. It is 500 times rarer than Parkinson's disease. Its effects can sometimes be severe enough to prevent patients from being able to perform daily functions. It is usually associated with structural brain lesions but can occur with metabolic abnormalities...

).

Presentation

Choreia is characterized by brief, quasi-purposeful, irregular contractions that are not repetitive or rhythmic, but appear to flow from one muscle to the next.

These 'dance-like' movements of choreia (from the same root word as "choreography") often occur with athetosis
Athetosis
Athetosis is a symptom characterized by involuntary convoluted, writhing movements of the fingers, arms, legs, and neck. Movements typical of athetosis are sometimes called athetoid movements. Lesions to the brain are most often the direct cause of the symptoms, particularly to thecorpus striatum...

, which adds twisting and writhing movements.

Causes

Choreia can occur in a variety of conditions and disorders.
  • Choreia is a primary feature of Huntington's disease
    Huntington's disease
    Huntington's disease, chorea, or disorder , is a neurodegenerative genetic disorder that affects muscle coordination and leads to cognitive decline and dementia. It typically becomes noticeable in middle age. HD is the most common genetic cause of abnormal involuntary writhing movements called chorea...

    , a progressive neurological disorder.
  • Twenty percent (20%) of children and adolescents with rheumatic fever
    Rheumatic fever
    Rheumatic fever is an inflammatory disease that occurs following a Streptococcus pyogenes infection, such as strep throat or scarlet fever. Believed to be caused by antibody cross-reactivity that can involve the heart, joints, skin, and brain, the illness typically develops two to three weeks after...

     develop Sydenham's chorea
    Sydenham's chorea
    Sydenham's chorea or chorea minor is a disease characterized by rapid, uncoordinated jerking movements affecting primarily the face, feet and hands. Sydenham's chorea results from childhood infection with Group A beta-hemolytic Streptococci and is reported to occur in 20-30% of patients with...

     as a complication.
  • Choreia gravidarum is rare type of choreia which is a complication of pregnancy
    Pregnancy
    Pregnancy refers to the fertilization and development of one or more offspring, known as a fetus or embryo, in a woman's uterus. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets...

    .
  • Choreia may also be caused by drugs (levodopa
    Levodopa
    L-DOPA is a chemical that is made and used as part of the normal biology of some animals and plants. Some animals including humans make it via biosynthesis from the amino acid L-tyrosine. L-DOPA is the precursor to the neurotransmitters dopamine, norepinephrine , and epinephrine collectively...

    , anti-convulsants, anti-psychotics), metabolic disorders, endocrine disorders, and vascular incidents.
  • Ataxia telangiectasia
    Ataxia telangiectasia
    Ataxia telangiectasia is a rare, neurodegenerative, inherited disease that affects many parts of the body and causes severe disability. Ataxia refers to poor coordination and telangiectasia to small dilated blood vessels, both of which are hallmarks of the disease...

  • Wilson's disease
    Wilson's disease
    Wilson's disease or hepatolenticular degeneration is an autosomal recessive genetic disorder in which copper accumulates in tissues; this manifests as neurological or psychiatric symptoms and liver disease...

    , a genetic disorder that leads to toxic levels of copper
    Copper
    Copper is a chemical element with the symbol Cu and atomic number 29. It is a ductile metal with very high thermal and electrical conductivity. Pure copper is soft and malleable; an exposed surface has a reddish-orange tarnish...

     in the body
  • McLeod syndrome
    McLeod syndrome
    McLeod syndrome is a genetic disorder that may affect the blood, brain, peripheral nerves, muscle and heart. It is caused by a variety of recessively-inherited mutations in the XK gene on the X chromosome...

    , a genetic disorder that may affect the blood, brain, peripheral nerves, muscle and heart. Common features include peripheral neuropathy, cardiomyopathy and hemolytic anemia. Other features include limb chorea, facial tics, other oral movements (lip and tongue biting), seizures, a late-onset dementia and behavioral changes.

Ballism

When choreia is serious, slight movements will become thrashing motions; this form of severe choreia is referred to as ballism. Walking may become peculiar, and include odd postures and leg movements. Unlike ataxia
Ataxia
Ataxia is a neurological sign and symptom that consists of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum...

 and dystonia
Dystonia
Dystonia is a neurological movement disorder, in which sustained muscle contractions cause twisting and repetitive movements or abnormal postures. The disorder may be hereditary or caused by other factors such as birth-related or other physical trauma, infection, poisoning or reaction to...

, which affect the quality of voluntary movements or parkinsonism
Parkinsonism
Parkinsonism is a neurological syndrome characterized by tremor, hypokinesia, rigidity, and postural instability. The underlying causes of parkinsonism are numerous, and diagnosis can be complex...

, which is a hindrance of voluntary movements, the movements of choreia and ballism occur on their own, without conscious effort.

Treatment

There is no standard course of treatment for choreia. Treatment depends on the type of choreia and the associated disease. Although there are many drugs that can control it, no cure has yet been identified.
| Choreia gravidarum  >
Form Treatment
>|-
| Huntington's
Huntington's disease
Huntington's disease, chorea, or disorder , is a neurodegenerative genetic disorder that affects muscle coordination and leads to cognitive decline and dementia. It typically becomes noticeable in middle age. HD is the most common genetic cause of abnormal involuntary writhing movements called chorea...

-related
dopaminergic
Dopaminergic
Dopaminergic means related to the neurotransmitter dopamine. For example, certain proteins such as the dopamine transporter , vesicular monoamine transporter 2 , and dopamine receptors can be classified as dopaminergic, and neurons which synthesize or contain dopamine and synapses with dopamine...

 antagonists
Receptor antagonist
A receptor antagonist is a type of receptor ligand or drug that does not provoke a biological response itself upon binding to a receptor, but blocks or dampens agonist-mediated responses...

, although treatment is largely supportive.
>-
| Sydenham's chorea
Sydenham's chorea
Sydenham's chorea or chorea minor is a disease characterized by rapid, uncoordinated jerking movements affecting primarily the face, feet and hands. Sydenham's chorea results from childhood infection with Group A beta-hemolytic Streptococci and is reported to occur in 20-30% of patients with...

 
Haloperidol, carbamazepine and valproic acid. Usually involves antibiotic
Antibiotic
An antibacterial is a compound or substance that kills or slows down the growth of bacteria.The term is often used synonymously with the term antibiotic; today, however, with increased knowledge of the causative agents of various infectious diseases, antibiotic has come to denote a broader range of...

 drugs to treat the infection, followed by drug therapy to prevent recurrence.
haloperidol
Haloperidol
Haloperidol is a typical antipsychotic. It is in the butyrophenone class of antipsychotic medications and has pharmacological effects similar to the phenothiazines....

, chlorpromazine
Chlorpromazine
Chlorpromazine is a typical antipsychotic...

 alone or in combination with diazepam
Diazepam
Diazepam , first marketed as Valium by Hoffmann-La Roche is a benzodiazepine drug. Diazepam is also marketed in Australia as Antenex. It is commonly used for treating anxiety, insomnia, seizures including status epilepticus, muscle spasms , restless legs syndrome, alcohol withdrawal,...

, also pimozide
Pimozide
Pimozide is an antipsychotic drug of the diphenylbutylpiperidine class. It was discovered at Janssen Pharmaceutica in 1963. It has a high potency compared to chlorpromazine . On a weight basis it is even more potent than haloperidol. It also has special neurologic indications for Tourette syndrome...

 can also be used.
>-
| Wilson's disease
Wilson's disease
Wilson's disease or hepatolenticular degeneration is an autosomal recessive genetic disorder in which copper accumulates in tissues; this manifests as neurological or psychiatric symptoms and liver disease...

 
chelating
Chelation
Chelation is the formation or presence of two or more separate coordinate bonds between apolydentate ligand and a single central atom....

agents
>-
|-
| Drug-induced choreia
>-
| Metabolic and endocrine-related choreias
Treated according to their causes.
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