Abstract 4370897: Sydenham Chorea as the Presenting sign of Rheumatic Heart Disease in a 12 Year old

Introduction: Sydenham chorea (SC) is a form of acquired chorea of childhood and is linked to Acute Rheumatic Fever (ARF), which is an autoimmune response to infection with Group A Streptococcus (GAS). Historically called St Vitus Dance, SC is reported in some patients with ARF and forms part of the major criteria for ARF in the Jones Criteria. Case Presentation: A 12 years old female presented with 10-day history involuntary movements. She suffered sudden, involuntary, irregular twitching of the right upper, lower limbs and facial mouthing or grimacing. She developed irritability, restlessness, easy crying, anger over minor events, deteriorating handwriting and slurring of speech. A thorough history was taken from patient and family, it was discovered that she had recurrent episodes of sore throat in the last one year, last episode being 1.5 months before the onset of chorea. Her throat infections were never treated by a qualified doctor. Physical examination revealed spooning sign of the hands, milkmaid grip sign, pronator sign of the arms and darting tongue sign. Tonsillar hypertrophy grade I was also noted. Pansystolic murmur was present over apex, 2D Echo was performed which showed moderate MR and mild AR, LVEF was 65%. Blood work showed highly elevated Anti Streptolysin O levels of >650 IU/ml. MRI of the brain didn’t detect any abnormality. Based on the history and findings, a diagnosis of Acute Rheumatic Fever with Sydenham chorea and valvular heart disease was made. The patient was started on Phenoxymethyl Penicillin, Carbamazepine and Pantoprazole after which her choreiform movements started to ameliorate significantly. Discussion: Despite progress in earlier diagnosis and treatment of streptococcal infection, Sydenham Chorea is still an essential feature of acute rheumatic fever in developing nations. The exact pathophysiology of SC hasn’t been understood, various theories have been suggested for the same. Most accepted theory of SC is an autoimmune one, which occurs in response to the group A streptococcal infection. These antibodies are created against streptococcal antigens and react with neuronal protein tissues particularly in the basal ganglia that account for choreiform movements made by SC patients. Carditis is another severe manifestation of ARF. The cardiac lesion frequently encountered in ARF is mitral regurgitation, aortic regurgitation is the other frequently observed valvular disease.