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Abstract Details

Characterization of Neurosarcoid Myelitis and Assessment of Treatment Response: A Multicenter Retrospective Study
Autoimmune Neurology
C12 - Autoimmune and Inflammatory Myelopathies (2:20 PM-2:25 PM)
P1 - Poster Session 1 (9:00 AM-5:00 PM)
046
To determine if initial treatment with corticosteroids plus steroid-sparing immunosuppressive therapy (IST) results in faster gadolinium contrast-enhancement resolution in neurosarcoid myelitis (NSM) than corticosteroid monotherapy (CSM).
Neurosarcoidosis is a rare cause of myelitis, treated initially with corticosteroids. Whether combination therapy of corticosteroids plus IST is more effective than CSM at disease onset is unknown.
We retrospectively reviewed cases of definite or probable NSM, defined by Neurosarcoidosis Consortium Consensus Group, in adults treated at six United States centers. We characterized clinicoradiographic features and treatment outcomes after NSM diagnosis. Treatment groups were defined as CSM, corticosteroids plus intermediate oral IST (methotrexate or mycophenolate mofetil), corticosteroids plus highly effective IST (cyclophosphamide/TNF-alpha inhibitors), or corticosteroids plus other. We hypothesized that initial treatment with corticosteroids and IST would result in faster gadolinium contrast-enhancement resolution (primary end point).
63 patients with NSM (32 female, 30 definite, median age 48) were identified. 86% had spinal cord enhancement on post-gadolinium T1 sequences (8% without enhancement, 6% without data). Time from symptom onset to treatment initiation varied from 11 days to 10 years (median 4 months). All but one patient received corticosteroids initially. 16/63 received corticosteroids alone. 29/63 received corticosteroids and subsequently IST (10 TNF-alpha inhibitor/cyclophosphamide, 10 mycophenolate/methotrexate, 9 other). Median time to IST initiation was 9.1 months (range 1 – 132). 16/63 were treated with initial IST (4 TNF-alpha/cyclophosphamide, 8 mycophenolate/methotrexate, 4 other). There was no significant difference in time to contrast-enhancement resolution between corticosteroids with/without subsequent IST versus corticosteroids + initial IST (Wilcoxon-rank sum test 0.93) or CSM versus corticosteroids + initial IST (Wilcoxon-rank sum test 0.97). Limitations are small sample size, variations in initiation of treatment and doses used, and intervals of clinical/radiographic follow-up.

Standardization of reporting and monitoring of treatment and outcomes is needed for patients with NSM to better assess optimal initial therapy plans.
Authors/Disclosures
Denis T. Balaban, MD (Massachusetts General Hospital)
PRESENTER 		The institution of Dr. Balaban has received research support from Biogen. The institution of Dr. Balaban has received research support from ArgenX.
Giovanna Manzano, MD (MGH Department of Neurology) Dr. Manzano has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for InfuCare Rx.
Ahya S. Ali, MD (Westchester Medical Center) Dr. Ali has nothing to disclose.
Eoin P. Flanagan, MBBCh, FAAN (Mayo Clinic) The institution of Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Flanagan 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. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. The institution of Dr. Flanagan has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Pharmacy times. The institution of Dr. Flanagan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. The institution of Dr. Flanagan has received research support from Viela Bio. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Roche. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has a non-compensated relationship as a Member of medical Advisory Board with The MOG Project that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Journal of The Neurologic Sciences that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Neuroimmunology Reports that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology, Neuroimmunology Neuroinflammation (N2) Journal that is relevant to AAN interests or activities.
Allen J. Aksamit, Jr., MD, FAAN (Mayo Clinic) Dr. Aksamit has nothing to disclose.
Vyanka Redenbaugh, MB BCh BAO (Mayo Clinic) Dr. Redenbaugh has nothing to disclose.
Stacey Clardy, MD, PhD, FAAN (University of Utah) Dr. Clardy has received personal compensation for serving as an employee of Veterans Health Administration (VHA). Dr. Clardy has received personal compensation for serving as an employee of University of Utah Health. Dr. Clardy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AstraZeneca/Alexion. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen/Horizon. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arialys. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. Dr. Clardy has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology/AAN Publications. The institution of Dr. Clardy has received research support from NIH/NINDS. The institution of Dr. Clardy has received research support from SRNA. The institution of Dr. Clardy has received research support from Alexion/AstraZeneca. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a AAN Summer Meeting CoDirector Travel and Lodging with AAN. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with U of Iowa, Miami, Stanford, Barrow, Beaumont Health, CCF, Emory, Penn State, Mayo Clinic, Walter Reed.
Rohini D. Samudralwar, MD (The University of Pennsylvania) Dr. Samudralwar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Samudralwar 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. Samudralwar has received personal compensation in the range of $500-$4,999 for serving as a Speaker with Multiple Sclerosis Association of America.
Paunel Agyei, MD Dr. Agyei has nothing to disclose.
Aram Zabeti, MD (University of Cincinnati/Dept of Neurology) Dr. Zabeti has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Zabeti has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Zabeti has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Octave. Dr. Zabeti has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion. Dr. Zabeti has received personal compensation in the range of $100,000-$499,999 for serving on a Speakers Bureau for Biogen. Dr. Zabeti has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for EMD-Serono. Dr. Zabeti has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Genentech. Dr. Zabeti has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Sanofi-Genzyme. Dr. Zabeti has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for Horizon. Dr. Zabeti has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Janssen . Dr. Zabeti has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for BMS. Dr. Zabeti has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for TG.
Robert Baughman, PhD (NINDS/NIH) No disclosure on file
Bart Chwalisz, MD (Massachusetts General Hospital, Department of Neurology) Dr. Chwalisz has nothing to disclose.
Michael Levy, MD, PhD, FAAN (Massachusetts General Hospital/Harvard Medical School) Dr. Levy has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubishi Pharma. Dr. Levy has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB Pharma. Dr. Levy has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi. Dr. Levy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Levy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. Dr. Levy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Levy has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. Dr. Levy has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Various law firms. The institution of Dr. Levy has received research support from National Institutes Health.
Shamik Bhattacharyya, MD, FAAN (Brigham and Women's Hospital) Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NeuroLambda. Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion Pharmaceuticals. Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Bhattacharyya has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Continuum. Dr. Bhattacharyya has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Wiley. Dr. Bhattacharyya has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Merck. The institution of Dr. Bhattacharyya has received research support from Alexion Pharmaceuticals. The institution of Dr. Bhattacharyya has received research support from National Institute of Health. The institution of Dr. Bhattacharyya has received research support from UCB. The institution of Dr. Bhattacharyya has received research support from Genentech. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care.