Sydenham Chorea

What is Sydenham chorea?

This neurological movement disorder happens in children with group A beta-hemolytic Streptococcus infection. It’s the same bacteria that causes strep throat and rheumatic fever. Your immune system’s response to the infection may affect your nervous system, causing the movement disorder.

How can Sydenham chorea affect my child’s well-being?

Chorea stems from the word “dance.” Children with this condition experience movement disorders that include rapid, involuntary, uncoordinated movements that resemble dancing. In severe cases, these movements disrupt daily life.

People who develop Sydenham chorea usually get it in childhood after having strep throat or rheumatic fever. It’s the most common form of acquired chorea.

How common is Sydenham chorea?

The condition is rare in North America and Europe due to the widespread, early use of antibiotics to treat sore throats. Sydenham chorea is more common in developing countries where the treatment practices vary.

Who is likely to get Sydenham chorea?

It’s likely to occur in children between 5 and 15 years old. It’s more common in people assigned female at birth (AFAB). Sydenham chorea rarely affects children younger than 5 or adults.

What are the potential complications of Sydenham chorea?

Children face a higher risk of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS). Whether the streptococcal infection truly causes PANDAS is still controversial. Some common symptoms described in children with PANDAS include:

• Behavioral changes and inattention.
• Obsessive-compulsive features.
• Tics.

Your immune system’s response to the streptococcal infection may affect other organs, including skin, joints and heart. You may develop conditions including:

• Arthritis.
• Endocarditis.
• Heart valve disease.

What causes Sydenham chorea?

It occurs when a group A beta-hemolytic Streptococcus infection triggers an abnormal immune system response. Instead of attacking the bacteria, your immune system attacks healthy brain tissue. The target of the attack is the area in your brain that controls movement. Some antibodies targeted to this brain region have been detected in people with Sydenham chorea.

What are the symptoms of Sydenham chorea?

Symptoms can happen anywhere in your or your child’s body. They may come on suddenly, especially when your child is tired.

Physical symptoms

• Involuntary brief random movements of your arms and body, which give an impression of restlessness.
• Clumsiness.
• Difficulty maintaining hand grip.
• Hand wringing.
• Restlessness.
• Slight grimacing.
• Stumbling and falling.

Speech issues

• Slurred speech.
• Vocal outbursts.

Cognitive and emotional symptoms

• Anxiety.
• Difficulty concentrating.

When do Sydenham chorea symptoms start?

Symptoms are delayed after an acute infection and may begin up to six months after your child has had strep throat or rheumatic fever.

How is Sydenham chorea diagnosed?

Healthcare providers ask about your child’s medical history, symptoms and potential exposure to group A beta-hemolytic Streptococcus. A blood test to detect strep antibodies may support the diagnosis. If your child tests positive for Sydenham chorea, they may also need a heart evaluation to check for signs of valve disease or endocarditis.

How is Sydenham chorea treated?

The therapies that are best for your child depend on symptom severity. Mild forms of the disease might not require treatment.

How is moderate to severe Sydenham chorea treated?

Medication may be beneficial in children with moderate to severe symptoms. Steroids help your body get rid of antibodies. Additional medications may also be necessary.

These drugs are primarily used to treat other medical conditions, but they’re also safe to take for Sydenham chorea. They include:

• Anticonvulsants: These drugs help by stabilizing nerve impulses. They include valproic acid and carbamazepine.
• Antipsychotics: Drugs such as haloperidol control tics and outbursts.
• Dopamine depleters: Tetrabenazine is a drug that treats involuntary movements.

How can I prevent Sydenham chorea?

Timely strep throat and rheumatic fever treatment may lower the risk of this condition.

If your child has had Sydenham chorea, being on antibiotics until adulthood can lower the risk of recurrent streptococcal infections, as well as the risk of heart disease. The long-term focus of treatment is to prevent permanent damage to heart valves due to heart disease.

What is the prognosis for children with Sydenham chorea?

Most children make a full recovery in three to six months. A small number of children continue experiencing symptoms for up to two years.

Symptoms can also return later in life, especially in people AFAB who take estrogen, birth control pills or get pregnant.

What is daily life like with Sydenham chorea?

Children with moderate to severe symptoms might not be able to perform everyday tasks. One of the challenges of living with Sydenham chorea is that symptoms can come on suddenly. They can also fluctuate throughout the day. Both situations can make it difficult to participate in regular childhood activities like going to school or spending time with friends.

A note from Cleveland Clinic

Sydenham chorea is an acquired neurological disorder that stems from a group A beta-hemolytic Streptococcus infection. It’s more common in developing countries where children may not receive antibiotic therapy for the infection. Even with successful treatment, a small number of children develop heart issues and other complications later in life. Most children make a full recovery and experience no complications.