A Rare Encounter: Sydenham Chorea with Elevated Interleukin 12 Levels Symptom in Responsive to Plasmapheresis and Immunotherapy

ObjectiveUnusual presentation of Sydenham chorea with high Interleukin 12 levels: successful treatment with plasmapheresis and immunotherapy. BackgroundSydenham chorea is a movement disorder characterized by emotional lability, hypotonia, and chorea with involuntary brief, random, and irregular movements of the limbs and face. Mild cases of Sydenham chorea without other manifestations of acute rheumatic fever may be mistakenly ascribed to behavioral or emotional disorders, Tourette syndrome, or clumsiness. ResultsA 22-year-old woman presented with involuntary brief, random, and irregular movements of the limbs for 2 months, worsening agitation, and slurred speech for 1 week. She had been streptococcus-positive recurrently since childhood and additional episodes occurred 2 months prior to the onset of her chorea symptoms. Positive tests including Throat culture grew Beta-hemolytic streptococcus, TTE revealed mild mitral valve regurgitation, hsCRP 3.6, and ANA 1:80. Other tests, including MRI brain, MRI cervical spine, Long-term video EEG, CSF tests, autoimmune and paraneoplastic panel, infection, and metabolic panel, were unremarkable. The patient's symptoms significantly improved with Plasmapheresis, steroid taper, Amantadine, and Penicillin V. She has been compliant with Penicillin since discharge and follow-up appointments showed a residual milkmaid sign in the right hand. TTE revealed possible mitral valve vegetation, hsCRP back to normal, and FCYTP panel in 6 months showed Interleukin 12 increased at 3.9, which is usually elevated in acute compared to persistent Sydenham chorea. ConclusionsThe full workup and early management are crucial for remarkable immunotherapeutic outcomes of Sydenham chorea.