Sydenham Chorea: Symptoms, Causes, Treatment

What are the symptoms of Sydenham chorea?

Sydenham chorea is a neurological disorder characterized by involuntary, rapid, and irregular movements. Symptoms often include:

• Involuntary Movements: These can include jerky, uncontrollable movements of the limbs, face, and trunk. The movements are often irregular and can vary in severity.
• Muscle Weakness: Difficulty with fine motor tasks, such as writing or buttoning a shirt, due to muscle weakness or lack of coordination.
• Emotional Changes: Individuals may experience mood swings, irritability, or emotional lability. Anxiety and emotional instability are also common.
• Coordination Problems: Difficulty with balance and coordination, which can affect walking and performing everyday tasks.
• Facial Grimacing: Involuntary facial movements or grimacing.
• Speech Difficulties: Problems with speech, including slurred speech or difficulty articulating words, can occur.
• Abnormal Postures: The affected individual might adopt unusual postures due to muscle contractions.
• Cognitive Changes: In some cases, there may be mild cognitive impairment or changes in attention and concentration.

Sydenham chorea is often associated with rheumatic fever, which typically follows a streptococcal infection like strep throat. The condition predominantly affects children and adolescents and may improve over time with appropriate treatment. If you suspect Sydenham chorea, it’s important to consult a healthcare provider for accurate diagnosis and management.

What are the causes of Sydenham chorea?

Sydenham chorea is primarily caused by an autoimmune reaction triggered by a previous streptococcal infection, such as strep throat. The underlying mechanisms include:

• Post-Streptococcal Autoimmune Response: The most common cause of Sydenham chorea is an autoimmune reaction following an infection with Group A Streptococcus bacteria. The body’s immune system, in response to the streptococcal infection, mistakenly attacks certain areas of the brain, particularly the basal ganglia, leading to the characteristic involuntary movements.
• Rheumatic Fever: Sydenham chorea is often associated with rheumatic fever, a complication of untreated or inadequately treated streptococcal infections. Rheumatic fever is itself an autoimmune condition that can affect the heart, joints, and other parts of the body. Sydenham chorea is one of the major manifestations of rheumatic fever.
• Genetic Susceptibility: There may be a genetic predisposition that makes some individuals more susceptible to developing Sydenham chorea after a streptococcal infection. Certain genetic factors could influence the immune system’s response to the infection.

The exact mechanism by which streptococcal infections lead to autoimmune reactions in Sydenham chorea is not fully understood, but it is believed to involve molecular mimicry, where antibodies produced against the streptococcal bacteria mistakenly target the brain’s own tissues.

What is the treatment for Sydenham chorea?

The treatment for Sydenham chorea focuses on managing the symptoms, addressing the underlying rheumatic fever, and supporting the overall well-being of the patient. Key aspects of treatment include:

• Medications:
• Anticonvulsants: Medications such as valproic acid or carbamazepine may help control the involuntary movements associated with Sydenham chorea.
• Neuroleptics: Drugs like haloperidol or risperidone may be used to reduce chorea symptoms.
• Corticosteroids: In some cases, corticosteroids like prednisone may be prescribed to reduce inflammation and autoimmune activity, particularly if symptoms are severe.
• Antibiotic Prophylaxis: To prevent recurrence of streptococcal infections and subsequent rheumatic fever, long-term antibiotic prophylaxis (e.g., penicillin) may be recommended.
• Supportive Therapies:
• Physical Therapy: To help with coordination and motor skills, physical therapy can be beneficial.
• Occupational Therapy: Helps with daily activities and fine motor skills that may be affected by the condition.
• Emotional Support: Counseling or support groups may be helpful for dealing with the emotional and psychological impact of the disorder.
• Regular Monitoring: Ongoing follow-up with healthcare providers to monitor the effectiveness of treatment, manage any side effects, and adjust therapy as needed.

Treatment plans are individualized based on the severity of symptoms and the overall health of the patient. Collaboration with a healthcare team, including neurologists and rheumatologists, ensures comprehensive management of the condition.