SYDENHAMS CHOREA ALONG WITH EVOLVING RHEUMATIC VALVULITIS

Manjusha Goel; LK. Senthil Kumar

doi:https://10.7199/ped.oncall.2014.11

Manjusha Goel, LK. Senthil Kumar.

Department of Pediatrics, Gandhi Medical College, Bhopal, India.

ADDRESS FOR CORRESPONDENCE
Dr. Manjusha Goel, G1/4 Gulmohar Colony, E-8, Bhopal, India. Email: manjushagoel@rediffmail.com

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A 5 years old boy presented with involuntary movements involving his limbs, fingers, toes and facial muscles for 4 days, severe enough to impair his daily activities such as brushing teeth. There was no history of fever and sore throat. On examination, he was alert and oriented. There was chorea involving all four limbs with writhing movements of his hands, facial twitching and lip smacking. Muscle tone, deep tendon reflexes and muscle power were all symmetrical and severely reduced and he could not speak. Gait was unsteady. Cardiovascular system (CVS) examination showed normal heart sounds with no heart murmur. Other systems were normal. There were no subcutaneous nodules or joint swelling. On CVS examination on 9th day, there was a grade 2/6 ejection systolic murmur in pulmonary area. On investigations, anti-streptolysin O (ASO) titre was 600 IU, erythrocyte sedimentation rate (ESR) was 60 mm/hr, throat swab showed no growth for streptococcus and electrocardiogram (ECG) showed prolonged PR interval. The heart murmur became pansystolic on day 14 of hospitalisation. Echocardiogram demonstrated the presence of rheumatic valvulitis. The left atrium was dilated and there was a mild to moderate degree of mitral incompetence. No pericardial effusion was detected. Treatment was started with IV crystalline penicillin and aspirin (80 mg/kg/day). Clinical improvement was observed two days after the commencement of haloperidol. He was discharged on aspirin, haloperidol and ranitidine along with monthly benzathine penicillin.

The incidence of rheumatic fever is higher in developing countries. The diagnosis of rheumatic fever is based on the Jones criteria. (1) Arthritis is the most common manifestation, present in 80% of patients. Carditis occurs in 40-75% of patients in the first 3 weeks of the illness. Death may occur in the acute phase. Erythema marginatum and subcutaneous nodules are rare, less than 10% of patients are affected. (2) Sydenham's chorea is the late manifestations of the rheumatic fever with incidence varying from 5-20% in various studies. (3,4) In a study done by Vijayalakshmi et al, 70% of patients with rheumatic chorea had echocardiographic evidence of valvular involvement although this had not been detected clinically. (5). Subclinical valvulitis in rheumatic chorea has also been observed by Panamota M et al. (6) In this child, the rheumatic valvulitis evolved over time though chorea was already present. Thus we conclude that all cases of chorea even as young as 5 years of age should undergo echocardiography as early as possible to prevent, detect and timely manage the consequences of acute rheumatic fever.

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References

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