Background: Rheumatic chorea is an uncommon but time-honored neurological manifestation of acute rheumatic fever that usually presents weeks to months after an untreated streptococcal pharyngitis. When it presents alone, that is, without other manifestations of the disease, it could easily be overlooked or delayed in its diagnosis. In such a scenario, it is uniquely presented as an isolated primary presentation of rheumatic fever, which underscores the challenge of diagnosis and the importance of early recognition. Case presentation: A 14-year-old boy presented with automatic, aimless, and arrhythmic movements of his limbs and face. There was no fever, rash, or joint pain in the preceding weeks. Neurological examination demonstrated choreiform movements typical of Sydenham’s chorea, and serology suggested a recent Group A streptococcal infection. Transthoracic echocardiography revealed mild mitral valve leaflet thickening with mild mitral regurgitation consistent with subclinical rheumatic carditis. The patient was treated with benzathine penicillin for eradication and long-term prophylaxis, and valproic acid for symptomatic control. Symptoms improved over weeks and follow-up assessments showed near resolution of valvular changes without development of clinically significant rheumatic heart disease. Conclusion: This case highlights that rheumatic chorea may occur as an isolated type of acute rheumatic fever. Recognition of such a presentation is important in making early diagnosis and treatment, which are essential to prevent long-term effects such as rheumatic heart disease.
Nashwan et al. (Wed,) studied this question.