Abstract Details

Title Utility of the CNS Demyelinating Disease Panel in Patients Presenting with Atypical Demyelinating Attacks

Topic Autoimmune Neurology

Presentation(s) P7 - Poster Session 7 (5:00 PM-6:00 PM)

Poster/Presentation
Number 8-019

Objective To evaluate predictors of a positive CNS demyelinating disease panel (CDDP) in patients with atypical demyelinating attacks at a tertiary neuroimmunology clinic.

Background CDDP is a serum cell-based assay for antibodies against aquaporin-4 water channel (AQP4-IgG) and myelin oligodendrocyte glycoprotein (MOG-IgG). CDDP is recommended for evaluating atypical demyelinating attacks not suggestive of MS, but its clinical utility has not been fully explored.

Design/Methods We retrospectively evaluated patients referred to a tertiary neuroimmunology clinic who were tested for CDDP at Mayo Clinic Laboratories as part of a workup for an atypical demyelinating attack.

Results From 2017 to 2024, 66 patients were tested for CDDP for an atypical demyelinating attack. Twenty-nine patients (43% of total) tested positive for MOG-IgG (including 8 patients deemed to have false positive antibody); 19 patients (28.4%) tested positive for AQP4-IgG (no false positives), and 18 patients (26.9%) tested negative for both. The rate of true positive panel was 60% with a sensitivity of 100% and specificity of 69%. Longitudinally-extensive optic neuritis predicted true positive MOG-IgG. Transverse myelitis, bowel dysfunction, tonic spasms, and spinal tremor predicted positive AQP4-IgG. Eye pain, focal dystonia, and a high IgG synthesis rate predicted a negative panel. Among patients presenting with atypical optic neuritis at testing (20, 29.9% of total), predictors of a true positive MOG-IgG test included steroid-responsiveness and normal spine MRI. Among patients presenting with atypical transverse myelitis (17, 25.8%), predictors of positive AQP4-IgG included bowel dysfunction and tonic spasms.

Conclusions Among patients presenting with atypical demyelinating attacks, the rate of true positive CDDP is high (60%) with high sensitivity and specificity. Among those, predictors of a negative test include painful optic neuritis, focal dystonia, and high IgG synthesis rate. Predictors of positive AQP4-IgG include transverse myelitis, tonic spasms, spinal tremor, and bowel dysfunction. Predictors of true positive MOG-IgG include longitudinally-extensive optic neuritis and steroid-responsiveness.

Authors/Disclosures
Manisha Ramprasad PRESENTER	Ms. Ramprasad has nothing to disclose.
Alessandro Serra, MD (University Hospitals Cleveland Medical Center)	Dr. Serra has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen Idec. Dr. Serra has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Bristol Myers Squibb.
Hesham A. Abboud, MD (University Hospitals Cleveland Medical Center)	Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. The institution of Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech . Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Horizon. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BMS. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alpine Pharma. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cycle Pharma. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Axonics. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen. Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Genentech. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for BMS. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Horizon. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for TG Therapeutics. The institution of Dr. Abboud has received research support from Genentech . The institution of Dr. Abboud has received research support from Novartis. The institution of Dr. Abboud has received research support from BMS. The institution of Dr. Abboud has received research support from Sanofi-Genzyme. The institution of Dr. Abboud has received research support from The Guthy-Jackson Charitable Foundation. The institution of Dr. Abboud has received research support from UCB. Dr. Abboud has received publishing royalties from a publication relating to health care.

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