Abstract Details

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

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (12:00 PM-1:00 PM)

Poster/Presentation
Number 025

Objective

Unusual presentation of Sydenham chorea with high Interleukin 12 levels: successful treatment with plasmapheresis and immunotherapy.

Background

Sydenham 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.

Design/Methods NA

Results A 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.

Conclusions

The full workup and early management are crucial for remarkable immunotherapeutic outcomes of Sydenham chorea.

Authors/Disclosures
Zhimin Xu, MD, PhD (Westchester Medical Center) PRESENTER	Dr. Xu has nothing to disclose.
Jon Rosenberg, MD	Dr. Rosenberg has nothing to disclose.
Robert Fekete, MD	Dr. Fekete has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Neurocrine Biosciences, Inc.. Dr. Fekete has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Acorda, Inc.. Dr. Fekete has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Abbvie. Dr. Fekete has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Teva Neuroscience, Inc.. Dr. Fekete has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Acadia Pharmaceutical, Inc.. Dr. Fekete has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Kyowa Kirin, Inc.. Dr. Fekete has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Adamas Pharmaceutical, Inc.. Dr. Fekete has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Neurocrine Biosciences, Inc.. Dr. Fekete has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Amneal Pharmaceuticals, Inc.. Dr. Fekete has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Acorda Therapeutics, Inc.. Dr. Fekete has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sunovion, Inc.. Dr. Fekete has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Medlink, Inc.. The institution of Dr. Fekete has received research support from Lundbeck. Dr. Fekete has received publishing royalties from a publication relating to health care. Dr. Fekete has a non-compensated relationship as a Neurological Working Group Member with Alliance for Patient Access that is relevant to AAN interests or activities.
Anila Thomas, MD	Dr. Thomas has nothing to disclose.

��ɫ��

��ɫ��