What are the other Names for this Condition? (Also known as/Synonyms)

• Juvenile Chorea
• Rheumatic Chorea
• St. Vitus Dance

What is Sydenham Chorea? (Definition/Background Information)

• Sydenham Chorea is considered a post-infectious autoimmune disorder that develops in children (predominantly girls). It is characterized by a rapid and involuntary jerking movements of the face, trunk, and limbs (also known as chorea)
• Sydenham Chorea follows rheumatic fever caused by Streptococcal throat infection, and may occur several weeks, months, or even a year after the initial throat infection
• Antibodies, which are generated in the body to clear the infection, begin destroying the body’s own cells (known as an autoimmune response) in certain genetically-predisposed individuals, resulting in Sydenham Chorea.
• In the brain, these autoantibodies attack the cells of the basal ganglia, leading to a set of neurological and behavioral symptoms. The basal ganglia region is involved in motor movement, coordination, and speech
• Apart from rapid and jerky movements, the symptoms of Sydenham Chorea may include speech and handwriting problems, walking difficulties, limp muscles, sore throat, fever, skin lesions, and heart murmurs
• Sydenham Chorea generally resolves on its own in a few weeks to months; no treatment is necessary in many individuals, unless the symptoms are severe or persistent
• The prognosis is generally good, since it usually resolves completely without presenting any complications. However, in one-third of the affected children, Sydenham Chorea may recur over the next 1-2 years

Who gets Sydenham Chorea? (Age and Sex Distribution)

• Sydenham Chorea is the most common acquired chorea in childhood
• The condition most commonly affects children between 5-15 years of age. Rare cases in children under 5 years of age and adults over 30 years have been recorded
• Although both genders are affected by Sydenham Chorea, girls are affected more than boys
• The condition occurs worldwide, without any racial or ethnic bias. But, the frequency of occurrence is higher in the developing countries than the developed countries

What are the Risk Factors for Sydenham Chorea? (Predisposing Factors)

The following factors may increase the risk for Sydenham Chorea:

• Age: Children aged between 5-15 years have a high risk
• Girls are at a higher risk for the condition than boys
• Living in overcrowded conditions, poor sanitation, etc.
• Poor hygiene
• A combination of attention-deficit hyperactive disorder (ADHD) and rheumatic fever
• Approximately, 25% of individuals with rheumatic fever are known to develop Sydenham Chorea

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Sydenham Chorea? (Etiology)

Sydenham Chorea is considered to be an autoimmune disorder. The sequence of events leading to Sydenham Chorea is as follows:

• Group A Streptococcus, a type of Gram-positive bacterium, infects a child.  This bacterium causes strep-throat, which is a common ailment during childhood. If the infection is not adequately treated, it can result in rheumatic fever
• Antibodies are generated in the body to clear the infection. These antibodies, generated against bacterial cells, begin destroying the cells of the individual (known as an autoimmune response)
• Such “autoantibodies” recognize specific proteins on certain types of cells and destroys them. The cells may include those of the kidneys, heart, joints, and brain
• The exact reason for this autoimmune response is not clearly understood. It is theorized that such a response occurs in certain genetically-predisposed individuals
• In the brain, the antibodies interact with the cells of basal ganglia, which is the site responsible for controlling motor movements and coordination. As a result, the affected individual develop the characteristic symptoms of Sydenham Chorea

What are the Signs and Symptoms of Sydenham Chorea?

The signs and symptoms of Sydenham Chorea may appear as early as a few weeks, or as late as a year, following a strep-throat (streptococcus infection). The type and severity of symptoms may vary among individuals. The onset of symptoms may be gradual or sudden.

The neurological signs and symptoms of Sydenham Chorea may include:

• Jerky movements of the hands, arms, face, trunk: These movements may increase during periods of stress, fatigue, or excitement, and subside during sleep
• Changes in handwriting
• Twitching of facial muscles; facial grimacing that appears like the child is “making faces”
• Low muscle tone
• Muscle weakness, affecting fine motor skills and causing clumsiness 
• Awkward gait due to uncoordinated movements
• Slurred speech

The behavioral signs and symptoms of Sydenham Chorea may include:

• Rheumatic Chorea may be associated with ADHD or OCD (obsessive compulsive disorder). Affected children may have obsessive or disturbing thoughts or actions
• Bouts of crying or laughing
• Cognitive disturbances
• Frequent mood swings
• Confusion
• Impairment in social relationships and functioning
• Personality changes

The signs and symptoms of Streptococcus sp. infection may include:

• Sore throat
• Fever
• Pharyngitis, which may cause sore throat and scratchy throat 
• Difficulty in swallowing

Signs and symptoms of rheumatic fever:

• Fever
• Joint pain
• Heart murmur (due to inflammation of the heart)
• Skin lesions and nodules

How is Sydenham Chorea Diagnosed?

The diagnosis of Sydenham Chorea is made on the basis of the following tests and exams:

• A thorough physical examination
• An assessment of signs and symptoms, with special note of characteristic “jerky” movements
• An evaluation of the affected individual’s medical history, particularly recent Streptococcus infection
• A throat swab to check for Streptococcus infection
• Blood tests to assess the following:
  • Complete blood count (CBC)
  • Erythrocyte sedimentation rate (ESR), to check for inflammatory activity in the body
  • C-reactive protein (CRP), which is an inflammatory marker
  • Anti-DNase B, to check for antibodies generated against a substance produced by group A Streptococcus bacteria
  • Anti-streptolysin antibody titer, to measure antibodies against group A Streptococcus bacteria
• Echocardiography to evaluate for carditis (inflammation of the heart muscle) 
• Magnetic resonance imaging (MRI) or computed tomography (CT) scan to rule-out other brain anomalies

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Sydenham Chorea?

Sydenham’s Chorea usually resolves without many complications. Rarely, the following complications may develop:

• Persistent, relapsing Sydenham Chorea despite treatment
• Recurrence of the condition several years after the initial incidence
• Endocarditis, or inflammation of the inner layer of the heart, which may necessitate surgery 

How is Sydenham Chorea Treated?

Generally, Sydenham Chorea resolves on its own without treatment. However, if neurological symptoms are severe and are not self-resolving then, the following treatment methods may be adopted:            

• Prescription of barbiturates, such as benzodiazepines and barbiturates, for sedation and rest
• Prescription of anti-epileptic drugs such as haloperidol or valproate 
• Oral steroid treatment for decrease immune response (not widely used)
• Intravenous injection of immunoglobulins to overcome immune response during initial stages of Sydenham Chorea; it has been used in clinical trials

In addition to the above, the following may be noted:

• For an active Streptococcus infection, antibiotics may be prescribed
• Children, who have had episodes of Sydenham Chorea, may be prescribed prophylactic antibiotics till they reach adulthood, in order to prevent future infections

How can Sydenham Chorea be Prevented?

• Sydenham Chorea may be preventable by seeking immediate medical care if the child develops sore throat, jerky movements, or any other signs of acute rheumatic fever
• If a child experiences an episode of Sydenham Chorea, the prophylactic use of penicillin is recommended for up to 10 years, for the prevention of further occurrences of the condition, which can potentially even damage the heart
• Regular medical screening at periodic intervals with tests, and physical examinations are recommended in children/adults who have been diagnosed with Sydenham Chorea

Also, the following methods may be adopted for prevention of Streptococcus infection that causes strep-throat and subsequent rheumatic fever:

• Frequent cleaning of hands with soap and water: Use of hand-sanitizer is not as good as washing one’s hands with soap and water; but, it is still a convenient method to reduce the chances of spread of infection
• Early diagnosis and treatment is an easy and appropriate method of avoiding spread of infection to others
• Covering one’s mouth while coughing or sneezing
• Avoiding sharing personal items, such as drinking glasses, utensils, or clothes, when infected
• Infected children should refrain from going to school (per advise of the healthcare provider)
• Children at school who may have been potentially exposed to Streptococcus infection may have to be monitored for any associated signs and symptoms
• Complete the course of antibiotics given for infection, as prescribed, in order to prevent the development of rheumatic fever (in future)
• Dispose the toothbrush being used, before completing the antibiotic therapy; Streptococcus bacteria can persist in a toothbrush and cause a reinfection

What is the Prognosis of Sydenham Chorea? (Outcomes/Resolutions)

• The prognosis for Sydenham Chorea is generally good in a majority; a full and complete recovery may take place within a time span of 3 weeks to 3 months
• However, in the affected individuals, there exists a 20% risk of recurrence

Additional and Relevant Useful Information for Sydenham Chorea:

Sydenham Chorea is named after Thomas Sydenham, a British doctor.