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Sydenhams Chorea is a neurologic movement disorder characterized by irregular, abrupt, relatively rapid involuntary movements of muscles of the face, neck, trunk, and arms and legs (limbs). Additional findings often include diminished muscle tone, 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.
In patients with Sydenham's chorea, choreic movements usually begin gradually, progressively worsening over a few weeks to a month. Associated findings may be extremely variable, ranging from relatively mild incoordination to severe disruption in conducting voluntary movements of multiple muscle groups, potentially affecting speech, arm movements, walking, and the ability to perform certain activities of daily living. In some patients, Sydenham's chorea may a self-limited condition, usually spontaneously resolving within about nine months to two years (about 50% of patients); therefore, treatment with certain medications may be restricted to patients with significantly impaired function resulting from severe chorea.
History of sore throat preceding, by several weeks, the appearance of Sydenham's chorea
Onset of uncontrollable movements
Movements are jerky and purposeless
Loss of fine motor control
Emotional lability with bouts of inappropriate crying or laughing
Any other signs of rheumatic fever
Sydenham chorea is one of the major signs of acute rheumatic fever. It is discussed here as a separate entity because it may be the only sign of rheumatic fever in some patients.
The movements seen in Sydenham chorea are involuntary, jerky, and purposeless. They are not rhythmic and occur sporadically in different muscle groups. For example, a sitting child might suddenly have an arm jerk upward followed by a leg extension, then a head nod. The movements occur in such a way that the child is constantly in motion and seems to be twitching everywhere.
Fine motor control becomes difficult, and handwriting may change dramatically.
Sydenham chorea occurs most frequently in prepubescent girls but may be seen in boys.
Suspected streptococcal infections must be treated. All the other manifestations of rheumatic fever, including Sydenham's chorea and excluding heart valve damage, remit with the acute disease and do not require treatment. Sydenham's chorea generally lasts for several months.
Most patients with Sydenham's chorea recover after a period of bed rest and temporary limitation of normal activities. In most cases the symptoms disappear gradually rather than stopping abruptly.
Most doctors recommend ongoing treatment with penicillin to prevent a recurrence of rheumatic fever or Sydenhams chorea, although there is some disagreement as to whether this treatment should continue for 5 years after an acute attack or for the rest of the patient's life. The penicillin may be given orally or by injection. Patients who cannot take penicillin may be given erythromycin or sulfadiazine.