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Sydenham Chorea – Causes, Symptoms and Treatment
Author: Corwin Brown | Posted: 02-05-2008 | Comments: 0 | Views: 7 | 
Sydenham's chorea is a neurologic movement disorder characterized by irregular, abrupt, relatively rapid involuntary movements (i.e., chorea) of muscles of the face, neck, trunk, and arms and legs (limbs). Additional findings often include diminished muscle tone (hypotonia), muscle weakness, and emotional and behavioral disturbances, particularly obsessive-compulsive behaviors. Sydenham's chorea most frequently occurs in children or adolescents between the ages of 5 to 15 following acute rheumatic fever (ARF). ARF is an inflammatory disease that develops subsequent to throat infection with certain strains of streptococcal bacteria (i.e., group A beta-hemolytic streptococci).
Chorea is an irregular, rapid, uncontrolled, involuntary, excessive movement that seems to move randomly from one part of the body to another. The affected child often appears fidgety or restless and unable to sit still. The word "chorea" comes from the Greek word for dance. The jerky movements of the feet or hands are often similar to dancing or piano playing. When chorea is severe, the movements may cause motion of the arms or legs that results in throwing whatever is in the hand or falling to the ground. Walking may become bizarre, with inserted excessive postures and leg movements.
Causes and Symptoms
Sydenham's is caused by certain types of streptococci called Group A beta-hemolytic streptococci or GAS bacteria. In general, streptococci are spherical-shaped anaerobic bacteria that occur in pairs or chains. GAS bacteria belong to a subcategory known as pyogenic streptococci, which means that the infections they cause produce pus. These particular germs seem to be able to create an immune response that attacks the body's own tissues along with the germs. Those tissues are joints, heart valves, skin, and brain.
Many patients suffer from strep throat, just before developing this new set of symptoms. They may also have joint pains without swelling, a condition known as arthralgia. Sydenham's chorea will appear as uncontrollable twitching or jerking of any part of the body that is worse when trying to repress it but disappears with sleep.
Psychological Symptoms
Psychological symptoms precede chorea and may be relapsing and remitting. They include:
· Emotional lability
· An obsessive trait
· Cognitive defects
· Personality change
Diagnosis
Because rheumatic fever is such a damaging disease, a complete evaluation should be done whenever it is suspected. This includes cultures for streptococci, blood tests, and usually an electrocardiogram (heartbeat mapping to detect abnormalities).
Treatment
There is no specific treatment for SD. For people with the mildest form, bed rest during the period of active movements is sufficient. When the severity of movements interferes with rest, sedative drugs, such as barbiturates or benzodiazepines, may be needed. Antiepileptic medications, such as valproic acid, are often prescribed. Doctors also recommend that children who have had SD take penicillin over the course of the next 10 years to prevent additional manifestations of rheumatic fever.
Anti-inflammatory medications such as aspirin or corticosteroids reduce inflammation to help manage acute rheumatic fever.
People who test positive for strep throat should also be treated with antibiotics. You may have to take low doses of antibiotics (such as penicillin, sulfadiazine, or erythromycin) over the long term to prevent the disease from returning.
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