Treatment Strategies in anti-myelin oligodendrocyte glycoprotein (MOG) associated Demyelinating Disease in a Pediatric Cohort (933)

Objective: To evaluate clinical features, including demographics, imaging characteristics, anti-MOG titer, treatment course, and treatment response in pediatric patients with MOG-antibody associated demyelinating diease. Background: Episodes of acute demyelinating syndromes occur in approximately 1 per 100,000 children per year and are typically monophasic. However, the presence of autoantibodies to MOG or aquaporin-4 help to predict relapse risk. Treatment strategies for neuromyelitis optica spectrum disorder have been heavily studied. Treatment strategies in MOG-antibody associated demyelinating disease remain largely anecdotal. Design/Methods: This is a non-randomized longitudinal case control study. Medical records of patients at a single academic center were reviewed and analyzed. Results: In this cohort of 13 children with a mean age of 13.2 years (5–18 years), 7 (54%) were female and 6 (46%) male. Antibody titers were 1:1000 in 1 (8%). On MRI, all subjects less than 10 years old had multifocal lesions. Children older than 10 presented with optic nerve lesion (5 of 9), spinal cord lesion (1 of 9), or multifocal (3 of 9). Acute treatments included IV steroids +/− plasmapheresis (9 received combination therapy). Seven of 13 had clinical relapses. There was no correlation between anti-MOG titer and relapse. Use of steroids did not correlate with risk of relapse (OR 1.2: 95% CI [0.1, 24.5]). Patients with relapses were started on long term immunotherapy: 4 on mycophenolate and 3 on rituximab. Tocilizumab was used for 1 patient with relapses on initial immunotherapy and was well tolerated. Conclusions: Prediction of relapse risk in anti-MOG seropositive patients is difficult. Long term immunotherapy is indicated in relapsing patients. Mycophenolate mofetil, rituximab, IVIG and tocilizumab have been used and are well tolerated. Long term data is necessary to better understand relapse risk and treatment efficacy. Disclosure: Dr. Fletcher has nothing to disclose. Dr. Wright has nothing to disclose. Dr. Sweeney has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Genentech and Novartis.