Chorea (or choreia, occasionally) is an abnormal involuntary
movement disorder, one of a group of neurological disorders called
dyskinesias. The term ''chorea'' is derived from the grc, χορεία ("dance"; see
choreia), as the quick movements of the feet or hands are comparable to dancing.
The term hemichorea refers to chorea of one side of the body, such as chorea of one arm but not both (analogous to
hemiballismus).
Presentation
Chorea is characterized by brief, semi-directed, irregular movements that are not repetitive or rhythmic, but appear to flow from one muscle to the next. These 'dance-like' movements of chorea often occur with
athetosis, which adds twisting and writhing movements. Walking may become difficult, and include odd postures and leg movements. Unlike
ataxia
Ataxia is a neurological sign consisting of lack of voluntary coordination of muscle movements that can include gait abnormality, speech changes, and abnormalities in eye movements. Ataxia is a clinical manifestation indicating dysfunction of ...
, which affects the quality of voluntary movements, or
Parkinsonism
Parkinsonism is a clinical syndrome characterized by tremor, bradykinesia (slowed movements), rigidity, and postural instability. These are the four motor symptoms found in Parkinson's disease (PD), after which it is named, dementia with Lew ...
, which is a hindrance of voluntary movements, the movements of chorea and
ballism occur on their own, without conscious effort. Thus, chorea is said to be a
hyperkinetic movement disorder. When chorea is serious, slight movements will become thrashing motions; this form of severe chorea is referred to as
ballism, or ballismus.
Causes
Huntington's disease
Huntington's disease
Huntington's disease (HD), also known as Huntington's chorea, is a neurodegenerative disease that is mostly inherited. The earliest symptoms are often subtle problems with mood or mental abilities. A general lack of coordination and an uns ...
is a neurodegenerative disease and most common inherited cause of chorea. The condition was formerly called Huntington's chorea but was renamed because of the important non-choreic features including
cognitive
Cognition refers to "the mental action or process of acquiring knowledge and understanding through thought, experience, and the senses". It encompasses all aspects of intellectual functions and processes such as: perception, attention, thought ...
decline and behavioural change.
Other genetic causes
Other genetic causes of chorea are rare. They include the classical Huntington's disease 'mimic' or
phenocopy syndromes, called
Huntington's disease-like syndrome
Huntington's disease-like syndromes (HD-like syndromes, or HDL syndromes) are a family of inherited neurodegenerative diseases that closely resemble Huntington's disease (HD) in that they typically produce a combination of chorea, cognitive declin ...
types 1, 2 and 3; inherited
prion disease
Prions are misfolded proteins that have the ability to transmit their misfolded shape onto normal variants of the same protein. They characterize several fatal and transmissible neurodegenerative diseases in humans and many other animals. It i ...
, the
spinocerebellar ataxias type 1, 3 and 17,
neuroacanthocytosis,
dentatorubral-pallidoluysian atrophy (DRPLA),
brain iron accumulation disorders,
Wilson's disease,
benign hereditary chorea,
Friedreich's ataxia
Friedreich's ataxia (FRDA or FA) is an autosomal-recessive genetic disease that causes difficulty walking, a loss of sensation in the arms and legs, and impaired speech that worsens over time. Symptoms generally start between 5 and 20 year ...
,
mitochondrial disease and
Rett syndrome.
Acquired causes
The most common acquired causes of chorea are
cerebrovascular disease and, in the developing world,
HIV infection—usually through its association with
cryptococcal disease.
Sydenham's chorea occurs as a complication of streptococcal infection. Twenty percent (20%) of children and adolescents with
rheumatic fever
Rheumatic fever (RF) is an inflammatory disease that can involve the heart, joints, skin, and brain. The disease typically develops two to four weeks after a streptococcal throat infection. Signs and symptoms include fever, multiple painful ...
develop Sydenham's chorea as a complication. It is increasingly rare, which may be partially due to penicillin, improved social conditions, and/or a natural reduction in the bacteria (Streptococcus) it has stemmed from. Psychological symptoms may precede or accompany this acquired chorea and may be relapsing and remitting. The broader spectrum of paediatric autoimmune neuropsychiatric disorders associated with streptococcal infection can cause chorea and are collectively referred to as
PANDAS.
Chorea gravidarum refers to choreic symptoms that occur during pregnancy. If left untreated, the disease resolves in 30% of patients before delivery but, in the other 70%, it persists. The symptoms then progressively disappear in the next few days following the delivery.
Chorea may also be caused by drugs (commonly
levodopa
-DOPA, also known as levodopa and -3,4-dihydroxyphenylalanine, is an amino acid that is made and used as part of the normal biology of some plants and animals, including humans. Humans, as well as a portion of the other animals that utilize -DOPA ...
,
anti-convulsants and
anti-psychotic
Antipsychotics, also known as neuroleptics, are a class of psychotropic medication primarily used to manage psychosis (including delusions, hallucinations, paranoia or disordered thought), principally in schizophrenia but also in a range of oth ...
s).
Other acquired causes include
CSF leak,
systemic lupus erythematosus
Lupus, technically known as systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary among people and may be mild to severe. Commo ...
,
antiphospholipid syndrome,
kappa light-chain monoclonal gammopathy of undetermined significance,
thyrotoxicosis,
polycythaemia rubra vera
Polycythemia vera is an uncommon myeloproliferative neoplasm (a type of chronic leukemia) in which the bone marrow makes too many red blood cells. It may also result in the overproduction of white blood cells and platelets.
Most of the he ...
,
transmissible spongiform encephalopathies,
coeliac disease and
gluten ataxia
Ataxia is a neurological sign consisting of lack of voluntary coordination of muscle movements that can include gait abnormality, speech changes, and abnormalities in eye movements. Ataxia is a clinical manifestation indicating dysfunction of ...
.
Treatment
There is no standard course of treatment for chorea. Treatment depends on the type of chorea and the associated disease. Although there are many drugs that can control it, no cure has yet been identified.
History
Historically, choreas like Huntington disease and Sydenham's chorea were called
Saint Vitus' dance, related to a series of
social phenomena of the same name.
See also
*
Choreoathetosis
*
Dancing mania
*
Tic
Notes
External links
*
{{Diseases of the nervous system
Extrapyramidal and movement disorders
Huntington's disease