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Sydenham's Chorea

RATIONALE: A major manifestation of acute rheumatic fever, chorea occurs most commonly between the ages of 7 and 14 years with the peak incidence at 8 years.

Stemming from the Greco- Latin word implying the act of dancing, the word chorea was first applied by Paracelsus to the frenzied movements of religious fanatics who during the middle ages journeyed to the healing shrine of St. Vitus. Osler defined Sydenham's Chorea as "an acute disease of childhood, rarely of adults, characterized by irregular movements, a variable amount of psychical disturbance, associated very often with arthritis or endocarditis."

Sydenham's Chorea is a disorder of the central nervous system characterized by emotional instability, purposeless movements, and muscular weakness. It is a major manifestation of acute rheumatic fever. Chorea occurs most commonly between the ages of 7 and 14 years with the peak incidence at 8 years. It is more common in girls than in boys. It is rare after puberty and exceedingly uncommon after the age of 20 years. In the 1950's chorea occurred in approximately 50% of cases of acute rheumatic fever. The incidence has declined substantially with chorea now being a component of less than 10% of cases of acute rheumatic fever.

Chorea may appear several weeks after an attack of acute rheumatic fever or it may be the initial symptom of a rheumatic episode. The onset is usually gradual. Typically the patient first becomes increasingly nervous and tends to have difficulty writing and may have reports of grimacing. Speech becomes indistinct and characteristic purposeless movements of the arms and legs develop. The movements are increased by effort, excitement, and fatigue but cease when the patient is asleep. Muscular weakness is common and the child may be unable to walk or even sit up.

In mild cases the movements can be exaggerated after periods of direct questioning or enforced quiet. When the patient talks there may be a "clucking sound" due to the sudden contraction of the tongue to the floor of the mouth. Characteristically if the patient is asked to extend the arms, hands, and fingers, flexion of the wrists and hyperextension of the metacarpophalangeal joints are observed. Other signs consist of an inability to hold the tongue still when it is protruded and spasmodic contractions of the hands when the patient intentionally grabs objects.

It is noteworthy that chorea may often be the only manifestation of acute rheumatic fever. Patients with Sydenham's Chorea not infrequently will not fulfill the Jones criteria. Other signs of rheumatic fever may be present and should be sought for carefully. Active carditis is found in a significant number of children whose initial complaint is chorea. Active arthritis however rarely occurs concurrently with chorea.

The diagnosis of Sydenham's Chorea largely lies on clinical grounds. Often there is minimal evidence of preceding streptococcal infection. In fact it is not infrequent for routine streptococcal serology including ASO titers, streptozyme, and anti- DNAase levels to all be normal.

The differential diagnosis must include entities such as familial chorea, CNS mass lesion, drugs or other toxins, and collagen vascular diseases. Sydenham's Chorea is usually a self limited condition and there is usually complete recovery. Mild cases may subside within a few weeks however the average case usually requires approximately 3 months for complete recovery. Relapses are not uncommon and occasionally signs of chorea may wax and wane for a year or more. No specific therapy aside from treatment of underlying streptococcal infection and rheumatic fever prophylaxis are required.