Prepared by: Dr. John Tharakan
Consultant Neurologist
Gleneagles Hospital Johor
What is Sydenham's Chorea?
Sydenham chorea is a neurological disorder that results from an autoimmune reaction to a group A streptococcal (GAS) infection. This leads to involuntary movements (also called chorea) of the face, limbs, and trunk. The condition can also cause emotional lability, muscle weakness, and difficulty with fine motor skills.
Most commonly seen in children between the ages of 5 and 15, Sydenham chorea is often a complication of rheumatic fever, a childhood infection caused by a streptococcal infection. While the condition typically resolves on its own within three to six months, treatment is important to prevent recurrence and manage symptoms.
Sydenham chorea is a movement disorder that primarily affects young children, particularly girls, and has a high recovery rate with appropriate treatment.
1. Is Sydenham Chorea Common Among Children In Malaysia?
Sydenham chorea, also known as St. Vitus’s dance is a complication of rheumatic fever, which itself is a childhood infection caused by certain streptococci bacteria. In a retrospective clinical study conducted at the University Malaya Medical Centre from 1967 to 1997, 313 patients with acute rheumatic fever were identified, of whom 12% had Sydenham chorea.
With the vast improvement in the health care system today, the incidence of rheumatic fever and Sydenham chorea has drastically decreased, and most children recover completely. Sydenham chorea typically resolves on its own within 3 to 6 months of initial onset, with cases rarely lasting longer than 1 year. Girls seem more prone to the condition than boys, and a majority of patients are in the five to 15-year-old age range.
2. What Causes Sydenham Chorea?
Chorea is a symptom of various underlying conditions rather than a specific disease. In the same way, a fever can happen for many different reasons. In most forms of chorea, there is an excess of dopamine, the main neurotransmitter in the basal ganglia, leading to abnormal, excessive movements. Some of the common causes of chorea that may be found in children are:
- Genetically determined conditions such as Huntington’s disease
- Lupus (systemic lupus erythematosus), an autoimmune disorder
- Overactivity of the thyroid gland (hyperthyroidism)
- A high blood sugar level (hyperglycaemia)
- A tumour or stroke affecting a part of the basal ganglia called the caudate nucleus.
While Sydenham chorea is often associated with rheumatic fever, which is triggered by streptococcal infection, other forms of chorea may be linked to these conditions.
3. What Are The Common Symptoms Parents Should Look Out For?
Sydenham chorea causes involuntary movements due to abnormal signals from the brain that affect motor control. Parents should watch for these common Sydenham chorea symptoms:
- Fidgeting, or Restlessness: These movements may occur at rest or during activity and often intensify during distracting tasks such as counting.
- Abnormal Movements: Involuntary movements can affect the entire body or be localized to one side, the face, hands, or arms.
- Muscle Weakness: Weakness in the muscles is another common symptom of Sydenham chorea, making it difficult for children to perform everyday activities.
These symptoms are typically seen in movement disorders, and parents should seek medical advice if they notice any of these signs in their children. Early intervention can help manage symptoms and improve the child’s quality of life.
4. What Are The Treatments For Sydenham Chorea?
In many cases, Sydenham chorea resolves on its own, as the symptoms are mild and typically resolve within 3 to 6 months. However, when the involuntary movements become severe and interfere with daily activities, medical intervention may be necessary.
Treatment options for Sydenham chorea include:
- Sedative Drugs: Medications like barbiturates or benzodiazepines may be prescribed to calm the hyperkinetic movements and reduce anxiety associated with the condition.
- Anti-Epileptic Medications: Drugs such as valproic acid can help manage the severity of the chorea and prevent further complications.
- Dopamine-Depleting Drugs: These medications work by reducing the excessive dopamine levels in the basal ganglia, which contribute to the abnormal motor movements.
The choice of treatment depends on the severity of the symptoms of Sydenham chorea and how much they affect the child's ability to function normally. Early intervention can help manage symptoms and prevent long-term issues.
Get Expert Care for Sydenham's Chorea at Gleneagles Hospital Johor
If your child is experiencing symptoms of Sydenham’s chorea, early diagnosis and treatment are crucial. Our expert team at Gleneagles Hospital Johor is here to provide comprehensive care and personalised treatment plans. Don’t wait, schedule a consultation today to ensure your child receives the best possible care for Sydenham’s chorea and other neurological conditions.
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