Sydenham's Chorea

Treatment

Adequate antibiotic therapy for children with streptococcal infections may help to prevent the onset of an initial attack of acute rheumatic fever (ARF). More specifically, such preventive therapy (i.e., primary prophylaxis) introduced up to about a week after the onset of streptococcal sore throat (pharyngitis) can prevent the onset of ARF.

The management of children with ARF may include restriction of normal activities and prompt administration of penicillin by mouth (orally) or intramuscular injection or other appropriate antibiotic therapies. Experts indicate that antibiotic therapy should be provided for all patients with ARF at the time of diagnosis since it may initially be difficult to confirm recent streptococcal infection due to the latent period, particularly in those with Sydenham's chorea. In addition, depending upon the severity of joint inflammation (arthritis) and heart inflammation (carditis), treatment may be provided with certain medications to help reduce pain and/or inflammation. For example, for those with arthritis, therapy may include codeine or salicylates, such as aspirin or other nonsteroidal anti-inflammatory drugs (NSAIDs). (As mentioned previously, early administration of salicylate therapy before diagnostic confirmation may confuse the diagnosis by preventing development of classic migratory polyarthritis. Thus, until the disease's clinical course has become evident, with subsequent diagnostic confirmation, experts recommend avoiding early therapy with anti-inflammatory agents and instead providing codeine or other similar agents for those with painful arthritis. On a cautionary note, the administration of aspirin or salicyliate in children may cause the rare but often fatal Reye syndrome.)

For patients with heart failure and other significant symptoms of carditis, therapy requires the administration of corticosteroid agents, such as prednisone. However, patients with mild carditis without signs of heart failure may receive sufficient benefit from salicylate therapy alone.

For those who receive salicylate therapy, blood levels and liver function must be regularly monitored (i.e., with blood and urine tests) to help reduce the possibility of salicylate toxicity, a condition that may be characterized by headache, rapid breathing (tachypnea), vomiting, irritability, reduced levels of sugar in the blood (hypoglycemia), and/or other findings. Steroid therapy should be limited in duration and dosage levels to help reduce adverse effects. In addition, such therapy should not be abruptly discontinued; rather, dosage levels should be gradually reduced (tapered down). As steroid dosage levels are tapered, physicians may recommend adding salicylates to help prevent a possible "rebound" of inflammatory symptoms. Salicylate therapy may be continued for approximately 2 to 4 weeks following the cessation of steroid administration.

For patients with heart failure, treatment may include the use of additional medications, such as diuretics to help eliminate excess fluid; certain heart medications that may strengthen contractions of the heart (cardiac glycosides, e.g., digitalis); and continued bed rest as required (although prolonged bed rest is typically unnecessary). Rarely, if such therapy is not effective, certain surgical measures may be required to replace or surgically repair damaged heart valves or to widen (dilate) narrowed valves through use of a balloon-tipped catheter (valvuloplasty).

As Sydenham's chorea may spontaneously resolve or not cause significant functional impairment, many experts indicate that treatment with certain medications, such as dopamine blockers (antagonists), should be avoided unless associated chorea is functionally disabling or associated with potentially violent flailing motions of the limbs that may result in self-injury. Dopamine antagonists, such as the antipsychotic agents haloperidol (Haldol®) or pimozide (Orap®), may have potentially severe adverse effects, including the development of tardive dyskinesia. First-line therapy with the anticonvulsant medication valproate sodium (Depakene®) may be beneficial for some patients with Sydenham's chorea. Pimozide is usually reserved for those patients who fail to respond to valproate or who present with severe forms (i.e., chorea paralytica). If these two options fail, the next steps may include immunomodulatory treatment, steroids, IV IgG, or plasmapheresis.

Experts indicate that patients who have had ARF or Sydenham's chorea should receive ongoing therapy to help prevent recurrences of rheumatic fever. Such preventive (i.e., secondary prophylactic) therapy may include intramuscular penicillin injections (benzathine penicillin G) every 21 days, daily penicillin by mouth (orally), or oral daily therapy with the antibacterial medication sulfadiazine or the antibiotic erythromycin (if other medications cannot be taken). Some experts indicate that prophylactic therapy should be lifelong for all those affected by ARF or chorea. Others recommend that therapy should be provided after an acute attack for 5 years or up to age 18 (whichever comes first) and should be longer only for those who have significant rheumatic valvular heart disease or an increased risk of reinfection (e.g., those who live in crowded conditions, health care professionals, teachers, etc.). Still others indicate that such secondary prophylaxis should be lifelong for all those with rheumatic heart disease. Individuals with rheumatic valvular disease are at an increased risk for developing bacterial infection of the heart valves and the lining of the heart chambers (endocarditis).