Rheumatic chorea What we need to know

Synonyms: Sydenham's chorea, minor chorea

Rheumatic or Sydenham's chorea is a later and sometimes the only manifestation of acute rheumatic fever and is the most common form of chorea acquired in childhood. Rheumatic (Sydenham) chorea is a condition characterized by involuntary, irregular movements of the muscles of the face and limbs, emotional lability, and muscle hypotonia.

The disease is caused by group A beta hemolytic streptococcus - the same pathogen that causes angina, gout, and rheumatoid arthritis.

Pathophysiology

Rheumatic chorea is an autoimmune movement disorder, in the pathogenesis of which infection caused by group A beta-hemolytic streptococcus plays the role of a trigger mechanism. Cross-reactive antibodies produced during the infectious process cause dysfunction of the basal ganglia of the brain and movement disorders. Studies have identified antibody target sites in the basal ganglia, damage to which disrupts neural circuits and causes symptoms.

In acute rheumatic fever, antibodies are produced against N-acetyl-beta-D-glucosamine, which is an important structural protein and is the immunodominant carbohydrate antigen in processes caused by group A beta-hemolytic streptococcus. These antibodies cause valvular heart lesions in rheumatic carditis.

Different subsets of antibodies to N-acetyl-beta-D-glucosamine correlate with the course of acute rheumatic fever.

In Sydenham's (rheumatic) chorea, the produced antibodies bind to the membrane proteins of nerve cells - lysogangliosides, which causes the initiation of a signaling cascade process. The target of these antibodies is also the intracellular protein tubulin, the interaction with which causes the activation of the pathogenic processes of rheumatic chorea.

reasons

Rheumatic (Sydenham) chorea develops in children aged 5-15 years. It is preceded by angina, rheumatic fever, or other streptococcal inflammation that causes an abnormal immune response and the development of antibodies that damage nerve cells (the basal ganglia of the brain). The disease is quite rare in developed countries, where streptococcal processes are treated with antibiotics in a timely manner. The frequency of the disease is higher in girls. Rheumatic chorea practically does not develop in children under 5 years of age and in adults.

Symptoms

Children who develop chorea rheumatic after a streptococcal infection are at increased risk of developing pediatric autoimmune disorders, the manifestations of which are:

• Behavioral and attention disorders
• Obsessions and compulsions
• tics

The developed immune response - antibodies to streptococcal infection may cause such injuries as:

Arthritis

Endocarditis

Valvular heart failure

• Involuntary rapid asynchronous movements of the limbs and facial muscles, giving the impression of restlessness
• tightness
• Difficulty holding hands
• Facial muscle contractions
• Stumbling or falling while walking
• Unclear, unclear speech
• vocal "explosions"
• Anxiety
• Decreased ability to concentrate

Symptoms of rheumatic chorea develop at least 6 months after the end of acute streptococcal infection.

Diagnosis

When diagnosing the disease, it is important to collect anamnestic data about the transmitted streptococcal infection. Detection of antibodies against streptococci confirms the diagnostics.

Laboratory diagnostics includes the following studies:

• General blood test
• Determination of erythrocyte sedimentation rate
• Rheumatoid factor
• Antibodies against citrullinated peptide – anti-CCP
• Antibodies against vimentin
• C-reactive protein
• Antinuclear antibodies ANA

In addition, cardiac ultrasound is important for screening for valvular damage and endocarditis.

Treatment

Treatment depends on the severity of the disease. Mild forms may not even require medical intervention.

In the case of moderate and severe manifestations of chorea, medical treatment is necessary:

Steroid therapy – It is necessary to reduce the number of antibodies and their damaging effects.

Anticonvulsants – Contributes to the stabilization of nerve impulse flows (valproic acid, carbamazepine).

antipsychotic (neuroleptic)  Medicines – to reduce tics and involuntary muscle contractions (haloperidol is the drug of choice).

Dopamine blocking drugs - To reduce involuntary movements (Tetrabenazine).

Prevention

Timely treatment of streptococcal processes reduces the risk of developing rheumatic chorea.

In the development of Sydenham (rheumatic) chorea, prolonged antibiotic therapy prevents valvular heart disease and endocarditis.

forecast

In most cases, recovery occurs within 3-6 months after the onset of chorea. Rarely, the condition may last up to 2 years.

Symptoms may recur later in adulthood due to pregnancy, contraceptive use, estrogen therapy, and other conditions.

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Resources

https://www.eular.org/world-arthritis-day-general-information

n.manjaparashvili@synevo.ge