Sydenham's chorea, also known as rheumatic chorea, is a disorder characterized by
rapid, uncoordinated jerking movements primarily affecting the face, hands and feet. Sydenham's chorea is an
autoimmune
In immunology, autoimmunity is the system of immune responses of an organism against its own healthy cells, tissues and other normal body constituents. Any disease resulting from this type of immune response is termed an " autoimmune disease" ...
disease that results from childhood infection with Group A beta-
haemolytic ''
Streptococcus
''Streptococcus'' is a genus of gram-positive spherical bacteria that belongs to the family Streptococcaceae, within the order Lactobacillales (lactic acid bacteria), in the phylum Bacillota. Cell division in streptococci occurs along a sing ...
''. It is reported to occur in 20–30% of people with acute
rheumatic fever
Rheumatic fever (RF) is an inflammation#Disorders, inflammatory disease that can involve the heart, joints, skin, and brain. The disease typically develops two to four weeks after a Streptococcal pharyngitis, streptococcal throat infection. Si ...
and is one of the major criteria for it, although it sometimes occurs in isolation. The disease occurs typically a few weeks, but up to 6 months, after the acute infection, which may have been a simple sore throat (
pharyngitis
Pharyngitis is inflammation of the back of the throat, known as the pharynx. It typically results in a sore throat and fever. Other symptoms may include a runny nose, cough, headache, difficulty swallowing, swollen lymph nodes, and a hoarse vo ...
).
Sydenham's chorea is more common in females than males, and most cases affect children between 5 and 15 years of age. Adult onset of Sydenham's chorea is comparatively rare, and the majority of the adult cases are recurrences following childhood Sydenham's chorea (although pregnancy and female hormone treatment are also potential causes).
It is historically one of the conditions called ''
St Vitus' dance''.
Signs and symptoms
Sydenham's chorea is characterized by the abrupt onset (sometimes within a few hours) of
neurological symptoms, classically
chorea
Chorea, or (rarely) choreia, () is an abnormal involuntary movement disorder, characterized by quick movements of the hands or feet. It is one of a group of neurological disorders called dyskinesias. The term ''chorea'' is derived , as the move ...
, which are non-rhythmic, writhing or explosive involuntary movements. Usually all four limbs are affected, but there are cases reported where just one side of the body is affected (''hemichorea''). Typical chorea includes repeated wrist
hyperextension
Motion, the process of movement, is described using specific anatomical terms. Motion includes movement of organs, joints, limbs, and specific sections of the body. The terminology used describes this motion according to its direction relativ ...
, grimacing, and lip pouting. The fingers can move as if playing the piano. There may be tongue
fasciculations
A fasciculation, or muscle twitch, is a spontaneous, involuntary muscle contraction and relaxation, involving fine muscle fibers. They are common, with as many as 70% of people experiencing them. They can be benign, or associated with more seriou ...
("bag of worms") and ''motor impersistence'', for example, the "milkmaid sign" (grip strength fluctuates, as if
hand milking a cow), or inability to sustain tongue protrusion (called
jack-in-the-box
A jack-in-the-box is a children's toy that outwardly consists of a Music Box, music box with a Crankset, crank. When the crank is turned, a music box mechanism in the toy plays a melody. After the crank has been turned a sufficient number of ti ...
tongue or
serpentine tongue, as the tongue slides in and out of the mouth), or eye closure.
There is usually a loss of
fine motor control, which is particularly obvious in
handwriting
Handwriting in Italian schools (XXth - XXIst century)
Handwriting is the personal and unique style of writing with a writing instrument, such as a pen or pencil in the hand. Handwriting includes both block and cursive styles and is separa ...
if the child is of school age. Speech is often affected (
dysarthria
Dysarthria is a speech sound disorder resulting from neurological injury of the motor component of the motor–speech system and is characterized by poor articulation of phonemes. It is a condition in which problems effectively occur with the ...
), as is walking; legs will suddenly give way or flick out to one side, giving an irregular
gait
Gait is the pattern of Motion (physics), movement of the limb (anatomy), limbs of animals, including Gait (human), humans, during Animal locomotion, locomotion over a solid substrate. Most animals use a variety of gaits, selecting gait based on s ...
and the appearance of skipping or dancing. Underlying the abnormal movements is often low tone (
hypotonia
Hypotonia is a state of low muscle tone (the amount of tension or resistance to stretch in a muscle), often involving reduced muscle strength. Hypotonia is not a specific medical disorder, but it is a potential manifestation of many different dis ...
) which may not become obvious until treatment is started to suppress the chorea.
The severity of the condition can vary from just some instability on walking and difficulty with handwriting, to the extreme of being wholly unable to walk, talk, or eat (''chorea paralytica'').
Movements cease during sleep.
It is a neuropsychiatric disorder, so besides the motor problems there is classically emotional lability (mood swings, or inappropriate mood), but also tics, anxiety, attention deficit etc. These can precede the motor symptoms.
Non-neurologic manifestations of acute rheumatic fever may be present, namely
carditis (up to 70% of cases, often subclinical, so echocardiography required),
arthritis
Arthritis is a general medical term used to describe a disorder that affects joints. Symptoms generally include joint pain and stiffness. Other symptoms may include redness, warmth, Joint effusion, swelling, and decreased range of motion of ...
,
erythema marginatum
Erythema marginatum (also known as chicken wire erythema) is an acquired skin condition which primarily affects the arms, trunk, and legs. It is a type of erythema (redness of the skin or mucous membranes) characterised by bright pink or red circ ...
, and subcutaneous
nodules
Nodule may refer to:
*Nodule (geology), a small rock or mineral cluster
*Manganese nodule, a metallic concretion found on the seafloor
*Nodule (medicine), a small aggregation of cells
*Root nodule
Root nodules are found on the roots of plants, p ...
.

Differentiating these signs from other involuntary movements such as
tics
A tic is a sudden and repetitive motor movement or vocalization that is not rhythmic and involves discrete muscle groups. Tics are typically brief and may resemble a normal behavioral characteristic or gesture.
Tics can be invisible to the obs ...
and
stereotypies can be difficult, and since these things are not uncommon they can potentially co-exist. Diagnosis is often delayed and attributed to another condition such as
tic disorder
Tic disorders are defined in the ''Diagnostic and Statistical Manual of Mental Disorders'' (DSM) based on type (motor or phonic) and duration of tics (sudden, rapid, nonrhythmic movements). Tic disorders are defined similarly by the World Health ...
or
conversion disorder
Conversion disorder (CD) was a formerly diagnosed psychiatric disorder characterized by abnormal sensory experiences and movement problems during periods of high psychological stress. Individuals diagnosed with CD presented with highly distressin ...
. The controversial
PANDAS
Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) is a controversial hypothetical diagnosis for a subset of children with rapid onset of obsessive-compulsive disorder (OCD) or tic disorders. Sy ...
(pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) hypothesis has overlapping clinical features, but Sydenham's chorea is one of the exclusion criteria. PANDAS can present with chorea but more typically there are
tics
A tic is a sudden and repetitive motor movement or vocalization that is not rhythmic and involves discrete muscle groups. Tics are typically brief and may resemble a normal behavioral characteristic or gesture.
Tics can be invisible to the obs ...
or
stereotypies with a psychological component (e.g.,
OCD).
Differential diagnosis
Other disorders that may be accompanied by chorea include
benign hereditary chorea,
bilateral striatal necrosis,
abetalipoproteinemia
Abetalipoproteinemia (also known as: Bassen–Kornzweig syndrome, microsomal triglyceride transfer protein deficiency disease, MTP deficiency, and betalipoprotein deficiency syndrome) is a disorder characterized by abnormal absorption of fat and ...
,
ataxia–telangiectasia
Ataxia–telangiectasia (AT or A–T), also referred to as ataxia–telangiectasia syndrome or Louis–Bar syndrome, is a rare, neurodegenerative disease causing severe disability. Ataxia refers to poor coordination and telangiectasia to small dil ...
,
biotin-thiamine-responsive basal ganglia disease (BTBGD),
Fahr disease,
familial dyskinesia–facial myokymia (Bird–Raskind syndrome) due to an
ADCY5 gene mutation,
glutaric aciduria,
Lesch–Nyhan syndrome,
mitochondrial disorders
Mitochondrial disease is a group of disorders caused by mitochondrial dysfunction. Mitochondria are the organelles that generate energy for the cell and are found in every cell of the human body except red blood cells. They convert the energy of ...
,
Huntington's disease
Huntington's disease (HD), also known as Huntington's chorea, is an incurable neurodegenerative disease that is mostly Genetic disorder#Autosomal dominant, inherited. It typically presents as a triad of progressive psychiatric, cognitive, and ...
,
Wilson disease,
hyperthyroidism
Hyperthyroidism is a endocrine disease in which the thyroid gland produces excessive amounts of thyroid hormones. Thyrotoxicosis is a condition that occurs due to elevated levels of thyroid hormones of any cause and therefore includes hyperth ...
,
lupus erythematosus
is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, ...
, pregnancy (
chorea gravidarum
Chorea gravidarum is a rare type of chorea which presents with involuntary abnormal movement, characterized by abrupt, brief, nonrhythmic, nonrepetitive movement of any limb, often associated with nonpatterned facial grimaces. It is a complication ...
),
drug intoxication
Substance intoxication is a transient condition of altered consciousness and behavior associated with recent use of a substance. It is often maladaptive and impairing, but reversible. If the symptoms are severe, the term "substance intoxication ...
and side effects of certain anticonvulsants (e.g.
phenytoin
Phenytoin (PHT), sold under the brand name Dilantin among others, is an anticonvulsant, anti-seizure medication. It is useful for the prevention of tonic-clonic seizures (also known as grand mal seizures) and focal seizures, but not absence se ...
) or psychotropic agents. Although some of these can similarly present in an acute way, there will typically be other neurological signs (such as ataxia or cognitive impairment), or other disease manifestations, or positive family history, which will help distinguish between them.
Pathology
One of the important manifestations of
acute rheumatic fever, Sydenham's chorea is believed to be caused by an
autoimmune
In immunology, autoimmunity is the system of immune responses of an organism against its own healthy cells, tissues and other normal body constituents. Any disease resulting from this type of immune response is termed an " autoimmune disease" ...
response following infection by
group A β-hemolytic streptococci.
Two cross-reactive streptococcal
antigens
In immunology, an antigen (Ag) is a molecule, moiety, foreign particulate matter, or an allergen, such as pollen, that can bind to a specific antibody or T-cell receptor. The presence of antigens in the body may trigger an immune response.
An ...
have been identified, the M protein and N-acetyl-beta-D-glucosamine, whereby infection leads to
autoantibodies
An autoantibody is an antibody (a type of protein) produced by the immune system that is directed against one or more of the individual's own proteins. Many autoimmune diseases (notably lupus erythematosus) are associated with such antibodies.
Pr ...
being produced against host tissues (
molecular mimicry) causing a variety of streptococcal related diseases including Sydenham's chorea but also
rheumatic heart disease
Valvular heart disease is any cardiovascular disease process involving one or more of the four valves of the heart (the aortic and mitral valves on the left side of heart and the pulmonic and tricuspid valves on the right side of heart). The ...
and
nephritic syndrome
Nephritic syndrome is a syndrome comprising medical sign, signs of nephritis, which is kidney disease involving inflammation. It often occurs in the Glomerulus (kidney), glomerulus, where it is called glomerulonephritis. Glomerulonephritis is ch ...
. Autoantibodies against basal ganglia proteins have been found in Sydenham's chorea but these are non-specific. Dopamine receptor autoantibodies have been reported to correlate with clinical symptoms. Whether these antibodies represent an
epiphenomenon
An epiphenomenon (plural: epiphenomena) is a secondary phenomenon that occurs alongside or in parallel to a primary phenomenon. The word has two senses: one that connotes known causation and one that connotes absence of causation or reservat ...
or are pathogenic, remains to be proven.
Epidemiology
Sydenham's chorea is primarily seen in children.
As with rheumatic fever, Sydenham's chorea is seen more often in less affluent communities, whether in the
developing world
A developing country is a sovereign state with a less-developed industrial base and a lower Human Development Index (HDI) relative to developed countries. However, this definition is not universally agreed upon. There is also no clear agreeme ...
or in
aboriginal communities in the
global North
Global North and Global South are terms that denote a method of grouping countries based on their defining characteristics with regard to socioeconomics and Global politics, politics. According to UN Trade and Development (UNCTAD), the Global S ...
. High rates of
impetigo
Impetigo is a contagious bacterial infection that involves the superficial skin. The most common presentation is yellowish crusts on the face, arms, or legs. Less commonly there may be large blisters which affect the groin or armpits. The les ...
are a marker for widespread streptococcal transmission.
In the UK, there are approximately 20 cases per year, according to a BPSU surveillance study.
Diagnosis
Chorea is distinctive, if the health care provider is familiar with it. The diagnosis is then made by the typical acute onset in the weeks following a sore throat or other minor infection, plus evidence of inflammation (raised
CRP and/or
ESR) and evidence of recent streptococcal infection.
To confirm recent streptococcal infection:
*
Throat culture
A throat culture is a laboratory diagnostic test that evaluates for the presence of a bacterial or fungal infection in the throat. A sample from the throat is collected by swabbing the throat and placing the sample into a special cup (culture) t ...
*
Anti-DNAse B titre (peaks at 8–12 weeks after infection)
*
Anti-streptolysin O titre (peaks at 3–5 weeks)
None of these tests are 100% reliable, particularly when the infection was some months previously.
Further testing is directed more towards alternative diagnoses and other manifestations of rheumatic fever:
*
Echocardiography
Echocardiography, also known as cardiac ultrasound, is the use of ultrasound to examine the heart. It is a type of medical imaging, using standard ultrasound or Doppler ultrasound. The visual image formed using this technique is called an ec ...
*
Electroencephalography
Electroencephalography (EEG)
is a method to record an electrogram of the spontaneous electrical activity of the brain. The biosignal, bio signals detected by EEG have been shown to represent the postsynaptic potentials of pyramidal neurons in ...
(EEG)
*
Lumbar puncture
*
Magnetic resonance imaging
Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and ...
or
computed tomography (CT) scan of the brain (alterations in
caudate nucleus
The caudate nucleus is one of the structures that make up the corpus striatum, which is part of the basal ganglia in the human brain. Although the caudate nucleus has long been associated with motor processes because of its relation to Parkinso ...
and
putaminal enlargement have been described in some patients)
Management

Management of Sydenham's chorea is based on the following principles:
* Eliminate the streptococcus – a course of penicillin is usually given at diagnosis to definitively clear any remaining streptococci (and avoid spread of a rheumatogenic clone), but there is no evidence that it makes any difference to the course of the illness.
* Treat the movement disorder
* Immunosuppression
* Prevention of relapses and further cardiac damage
* Manage the disability -
Occupational therapy
Occupational therapy (OT), also known as ergotherapy, is a healthcare profession. Ergotherapy is derived from the Greek wiktionary:ergon, ergon which is allied to work, to act and to be active. Occupational therapy is based on the assumption t ...
and
physiotherapy
Physical therapy (PT), also known as physiotherapy, is a healthcare profession, as well as the care provided by physical therapists who promote, maintain, or restore health through patient education, physical intervention, disease preventio ...
are useful for maintaining function and muscle tone.
Treatment of chorea
Treatment with
sodium valproate is effective for controlling symptoms, but it does not speed up recovery.
Haloperidol
Haloperidol, sold under the brand name Haldol among others, is a typical antipsychotic medication. Haloperidol is used in the treatment of schizophrenia, tics in Tourette syndrome, mania in bipolar disorder, delirium, agitation, acute psychos ...
was used previously, but caused serious side effects e.g.
tardive dyskinesia
Tardive dyskinesia (TD) is an iatrogenic disorder that results in involuntary repetitive body movements, which may include grimacing, sticking out the tongue or smacking the lips, which occurs following treatment with medication. Additional mo ...
. Case reports exist to support
carbamazepine
Carbamazepine, sold under the brand name Tegretol among others, is an anticonvulsant medication used in the treatment of epilepsy and neuropathic pain. It is used as an adjunctive treatment in schizophrenia along with other medications and as ...
and
levetiracetam
Levetiracetam, sold under the brand name Keppra among others, is a novel antiepileptic drug. (medication) used to treat epilepsy. It is used for Focal seizure, partial-onset, Myoclonic epilepsy, myoclonic, or tonic–clonic seizures, and is ta ...
; other drugs tried include
pimozide
Pimozide (sold under the brand name Orap) is a neuroleptic medication, drug of the diphenylbutylpiperidine class. It was discovered at Janssen Pharmaceutica in 1963. It has a high potency compared to chlorpromazine (ratio 50-70:1). On a weigh ...
,
clonidine
Clonidine, sold under the brand name Catapres among others, is an α2A-adrenergic receptor agonist medication used to treat high blood pressure, attention deficit hyperactivity disorder (ADHD), drug withdrawal (e.g., alcohol, opioids, or nic ...
, and
phenobarbitone.
Immunosuppression
Immunosuppression is used inconsistently in Sydenham's chorea. The model of an autoimmune disorder would support its use. One randomized controlled trial of steroids from Paz, Brazil in 2006 (22 cases) showed remission reduced to 54 days from 119 days. Various other reports of use of oral or IV steroids from Israel, Italy and Brazil.
Immunoglobulin has been used in Holland and South Africa. Some improvement can be seen within a few days of IV steroids. In Italy, prednisolone reduced average duration of symptoms from 9 weeks to 4 weeks, and these were severe cases.
South African group found less neuropsychiatric complications at 6 months with IVIG treatment (IVIG preferred due to fear of TB reactivation).
Relapse prevention
Penicillin prophylaxis is essential to treat cardiac features of rheumatic fever, even if subclinical (American Heart Association guideline). If there are not features to warrant a diagnosis of rheumatic fever, it is arguable whether cardiac risk justifies prophylaxis or not; however, it is likely to reduce recurrence.
Other treatments
There are several historical case series reporting successful treatment of Sydenham's chorea by inducing fever.
Prognosis
Symptoms usually get worse over the course of two weeks, then stabilize, and finally begin to improve.
Motor problems, including chorea, settle within an average of 2–3 months.
Recurrence is seen in 16–40% of cases. It is sometimes but not always associated with a rise in ASO titre or other evidence of new streptococcal infection. Recurrence is more likely with poor compliance with penicillin prophylaxis. It is more likely if there is failure to remit within 6 months of onset. Recurrence is associated in women with pregnancy (''chorea gravidorum'') and with female hormone treatment, although the onset of chorea can be delayed by months or more.
Intramuscular penicillin given every 2–3 weeks is superior to a 4 weekly regime for preventing relapse, but a risk assessment may conclude that twice daily oral penicillin is sufficiently effective, less painful for the child, and less demanding on the family.
Higher recurrence rates are seen with the longest follow up – relapse can be seen 10 years or more after the initial episode, so might be underestimated by series with shorter follow up.
Recurrence is usually only chorea, even if the original case was associated with rheumatic fever. There are two total reports of heart disease worsening after recurrence of chorea. The Thailand study also had 2 cases where carditis, which had improved after initial diagnosis, came back again. Some suggest that recurrent chorea is a different disease altogether.
10% reported long-term tremor in one study (10 years follow up). Long term neuropsychiatric difficulties are increasingly recognized (49 studies so far, especially
obsessive-compulsive disorder but also
attention-deficit hyperactivity disorder
Attention deficit hyperactivity disorder (ADHD) is a neurodevelopmental disorder characterised by symptoms of inattention, hyperactivity, impulsivity, and emotional dysregulation that are excessive and pervasive, impairing in multiple con ...
,
affective disorders,
tic disorders, executive function disturbances, psychotic features, and language impairment).
Heart involvement improves in about a third of cases (whether silent or not).
History
The incidence of acute rheumatic fever and rheumatic heart disease is not declining. Recent figures quote the incidence of Acute Rheumatic Fever as 0.6–0.7/1,000 population in the United States and Japan compared with 15–21/1,000 population in Asia and Africa.
The prevalence of Acute Rheumatic Fever and Sydenham's Chorea has declined progressively in developed countries over the last decades.
Etymology
It is named after British physician
Thomas Sydenham
Thomas Sydenham (; 10 September 1624 – 29 December 1689) was an England, English physician. He was the author of ''Observationes Medicae'' (1676) which became a standard textbook of medicine for two centuries so that he became known as 'The ...
(1624–1689).
The alternative eponym, "Saint Vitus Dance", is in reference to
Saint Vitus
Vitus (), whose name is sometimes rendered Guy or Guido, was a Christian martyr from Sicily. His surviving hagiography is pure legend. The dates of his actual life are unknown.Basil Watkins, ''The Book of Saints: A Comprehensive Biographical ...
, a Christian saint who was persecuted by
Roman
Roman or Romans most often refers to:
*Rome, the capital city of Italy
*Ancient Rome, Roman civilization from 8th century BC to 5th century AD
*Roman people, the people of Roman civilization
*Epistle to the Romans, shortened to Romans, a letter w ...
emperors and died as a martyr in AD 303. Saint Vitus is considered to be the
patron saint
A patron saint, patroness saint, patron hallow or heavenly protector is a saint who in Catholicism, Anglicanism, Eastern Orthodoxy or Oriental Orthodoxy is regarded as the heavenly advocate of a nation, place, craft, activity, class, clan, fa ...
of dancers, with the eponym given as homage to the manic dancing that historically took place in front of his statue during the feast of Saint Vitus in Germanic and Latvian cultures.
References
Further reading
*
External links
Sydenhams Chorea Associationfor patient & family support
research from Brazil's
Universidade Federal de Minas Gerais
The Federal University of Minas Gerais (, UFMG) is a federalIn the Brazilian Higher Education context, ''Federal'' does not mean ''collegiate'' (even though most Federal Universities in Brazil enjoy a similarly collegiate system), but it means ...
(UFMG)
{{DEFAULTSORT:Sydenham's Chorea
Neurological disorders