Rare Recurrence of Sydenham Chorea in an Adult: A Case Report

Abstract

Background: Sydenham chorea is thought to be an autoimmune condition that usually develops following a group A beta-hemolytic streptococcal infection.The onset of Sydenham chorea in adults is rare and most of the adult cases usually are secondary to recurrence following childhood illness. Risk factors for chorea recurrence include irregular antibiotic prophylactic use, failure to reach remission within 6 months, and symptom persistence for longer than a year.

Case presentation: A 27-year-old young adult Ethiopian female patient with chronic rheumatic valvular heart disease for the last 8 years experienced repetitive uncontrollable movements of her extremities and torso for three years prior to her current visit. Physical examination was significant for holosystolic murmur at the apical area radiating to the left axilla and choreiform movements apparent on all limbs and trunk. Investigations were significant for mildly raised ESR, echocardiography findings of thickened mitral valve leaflets and severe mitral regurgitation. She was successfully treated with valproic acid and the frequency of penicillin injection was made every 3 weeks with no recurrence for the first 3 months follow-up period.

Conclusion: We believe that this is the first case report of adult onset recurrent Sydenham chorea (SC) from a resource-limited setting. Though Sydenham chorea and its recurrence is rare in adults, it should be considered in adults after ruling out other competing differential diagnoses. Because of the lack of evidence on treatment of such rare cases, individualized mode of therapy is advised. Valproic acid is preferred for symptomatic treatment and more frequent benzathine penicillin G injections, for example every three weeks, may help in the prevention of recurrence of Sydenham chorea.

Keywords: Sydenham chorea; recurrence; rheumatic fever.

Figure 1

Transthoracic echocardiography from a patient with rheumatic valvular heart disease and recurrent Sydenham chorea in parasternal long axis view. Mitral valve leaflets are thickened (arrows).