Objective: Sydenham’s Chorea (SC) is a predominantly female childhood disorder characterized by irregular, fluid-like, involuntary movements. Here we present a case of isolated Sydenham’s chorea in an atypical epidemiological group.

Background: A 33 year-old male with a history of mild polycythemia presents to the neurology clinic to discuss persistent, sharp headaches with numbness radiating down the body.  On exam he has a soft stuttering voice, resting right upper extremity (RUE) tremor, diffuse hyperreflexia with a positive RUE Hoffman’s sign, and 2 beats of lower extremity clonus. He is sent to the Emergency Department (ED) for relief of headache with subsequent negative brain imaging, however, he is noted to have bizarre asymmetric fluid movements of his head and extremities. He developed stiffness and tightening of his extremities with inability to suppress these movements purposefully, while distracted, or with gait. He eventually started to develop paranoid delusions, emotional lability, and response to external stimuli. The patient underwent extensive multidisciplinary evaluation. High dose IV steroids were initiated early in the course with extensive serum and CSF analysis for a differential involving multiple variants of autoimmune encephalitis, polycythemia vera, and neuroacanthocytosis. Based on poor response to Steroids, he was started on a trial of Intravenous Immunoglobulin (IVIG). He began demonstrating some response to IVIG, while extensive workup was positive for Antistreptolysin O (ASO) and tested negative for acanthocytes, primary polycythemia, and other extensive etiologies. Throat culture was negative, but based on clinical course and sufficient clinical findings, diagnosis of Sydenham’s Chorea was made. After receiving Benzathine and full IVIG course, he had notable improvement in choreatic movements and psychiatric symptoms.

Method: NA

Results: NA

Conclusion: Sydenham’s Chorea is known as a childhood disorder established throughout the current literature. It is thought to be an inappropriate autoimmune response to initial Strep infection. This concept, however, does not necessarily always imply development of movement symptoms within a set period after initial infection, or in conjunction with rheumatic fever. This case serves as an example, among other more newly noted cases, of adult onset SC without clearly defined Strep A infection or Rheumatic Fever to re-evaluate the epidemiology and pathophysiology of SC.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/a-dance-with-sydenham-its-never-too-late-to-tango/