Abstract Details

Title Characterization of Neurosarcoid Myelitis and Assessment of Treatment Response in a Single-Hospital System.

Topic Autoimmune Neurology

Presentation(s) P6 - Poster Session 6 (5:30 PM-6:30 PM)

Poster/Presentation
Number 9-001

Background Neurosarcoid myelitis (NSM) is a rare inflammatory cause of myelopathy, usually treated with corticosteroids. Whether combination therapy of corticosteroids plus other IST at disease onset yields improved outcomes is unknown.

Objective

To investigate whether combination therapy of corticosteroids plus other immunosuppressive therapy (IST) at disease onset of neurosarcoid myelitis achieves radiographic resolution of disease (primary endpoint) faster than corticosteroid monotherapy.

Design/Methods We retrospectively reviewed cases of definite or probable NSM in adults treated within the Mass General Brigham system (Boston, USA) from January 1, 1970 through December 31, 2019. We characterized the clinical and radiographic features, and treatment outcomes of adults with NSM. Treatment groups were defined as corticosteroid monotherapy, corticosteroids plus intermediate oral IST (methotrexate or mycophenolate mofetil), corticosteroids plus highly effective IST (cyclophosphamide or infliximab), or corticosteroids plus other.

Results

We identified 13 patients with probable NSM (7 female, median age 42) with median EDSS 4 and median mRS 1 at diagnosis. CSF pleocytosis and elevated protein were present in 50% and 42%, respectively. Angiotensin-converting enzyme levels were normal in serum in 77% and normal in CSF in 8 of 8 tested patients. On MRI, all had T2 hyperintensity and 91% had parenchymal spinal cord enhancement. Duration between symptom onset to treatment initiation varied from 11 days to 4 years (median 5 months). All patients received corticosteroids initially, and seven of 13 received both corticosteroids and additional IST.

Conclusions There were no significant differences in time to contrast-enhancement resolution (Wilcoxon-rank sum test 0.70) and no differences in time to radiographic relapse (Fisher’s exact test p=0.43) by treatment group. Limitations of this study are small sample size, variable time to treatment, and variable interval of radiographic follow-up. This study supports a need for more standardized reporting and investigation of treatment effect on clinical and radiographic outcomes in a larger sample size.

Authors/Disclosures
Giovanna Manzano, MD (MGH Department of Neurology) PRESENTER	Dr. Manzano has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for InfuCare Rx.
Denis T. Balaban, MD (Massachusetts General Hospital)	The institution of Dr. Balaban has received research support from Biogen.
Ahya S. Ali, MD (Westchester Medical Center)	Dr. Ali has nothing to disclose.
Brian C. Healy	The institution of Mr. Healy has received research support from Analysis Group. The institution of Mr. Healy has received research support from Bristol-Myers Squibb. The institution of Mr. Healy has received research support from Verily Life Sciences. The institution of Mr. Healy has received research support from Novartis. The institution of Mr. Healy has received research support from Merck Serono. The institution of Mr. Healy has received research support from Genzyme.
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 on a Scientific Advisory or Data Safety Monitoring board for Alexion Pharmaceuticals. 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 $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. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care.