82. Sydenham’s Chorea
Definition
Sydenham’s chorea (SC) is a self-limiting disorder most often seen in children from 5 to 15 years of age. It may also manifest during pregnancy. This disease is often linked with rheumatic fever and is marked by involuntary movements that can become severe enough over time to affect all motor activities. Sydenham’s chorea is also known as St. Vitus’ dance and rheumatic chorea.
Incidence
The frequency of SC is related to the incidence of rheumatic fever. SC is estimated to occur in 10% to 20% of patients with rheumatic fever. In the United States, the incidence of rheumatic fever is 0.5:100,000 to 2:100,000. The incidence of SC would be estimated to be about 0.05:100,000 to 0.4:100,000. Internationally, the incidence of rheumatic fever is much higher in developing and underdeveloped countries where antibiotic treatments are much less available. Therefore the incidence of SC is higher in these countries.
Etiology
The underlying cause of SC is infection by the Lancefield group A β-hemolytic streptococcal organism, which apparently contributes to the development of antineuronal antibodies. The antineuronal antibodies cross-react with an unknown epitope in basal ganglia neurons.
Signs and Symptoms
• Deterioration of handwriting
• Emotional lability
• Facial grimacing
• Hypotonia
• Involuntary movements
• Irritability
• Muscle weakness
• Restlessness
• Slow or slurred speech
Medical Management
Sydenham’s chorea is usually a self-limiting disorder. The patient typically “outgrows” the disorder. Treatment is generally reserved for patients in whom the chorea becomes severe enough to interfere with daily functioning. Choreiform movements may be controlled with valproic acid; improvement is usually seen within 7 days of treatment initiation. Carbamazepine, an anticonvulsant, and diazepam may also produce desired results. Choreiform movements can also be controlled using dopaminergic blockers and depletors; however, their use has been constrained by the significant side effects they are known to produce.
Complications
• Cardiac conduction defects secondary to rheumatic fever
• Choreoathetosis
• Endocarditis
• Relapse of streptococcal infection
• Rheumatism
Anesthesia Implications
The close association of SC with a history of rheumatic fever should cause the anesthetist to focus on cardiac abnormalities that may have been produced by the streptococcal infection. The patient should receive a thorough preoperative cardiac evaluation, including a 12-lead electrocardiogram, even though the patient is younger than 20 years of age.
The patient should also receive prophylactic antibiotic coverage as a preventive measure related to the history of β-hemolytic streptococcal infection and rheumatic fever. The patient’s medication profile or history should be reviewed. The patient with SC may be undergoing treatment with corticosteroids, barbiturates, phenothiazines, and/or antiparkinsonian medications such as levodopa. Perioperative continuation of corticosteroids is particularly important because of the possible adrenal suppression long-term corticosteroid use can produce.
Administration of anesthesia to a patient with SC has been a rare event. Because anesthetic experience with SC patients is so limited, no technique or agent has been specifically recommended or contraindicated. The primary anesthetic concerns involve potential drug interactions from medications being used for the patient’s symptomatic treatment.
