Hacohen Y, et al. - Children with relapsing Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs)–associated disease were investigated for the clinical phenotypes, treatment responses, and outcomes. Observations revealed that disease-modifying drugs (DMDs) were not associated with clinical improvement in these children despite being commonly used to treat patients with multiple sclerosis. However, azathioprine, mycophenolate mofetil, rituximab, and particularly intravenous immunoglobulins were associated with a reduction in relapse frequency. Therefore to optimize immune treatment, a correct diagnosis of relapsing MOG-Ab–associated disorders seems important.

Methods

• From January 1, 2014, through December 31, 2016, researchers prospectively collected demographic, clinical, and radiologic data from 102 patients from 8 countries of the EU Paediatric Demyelinating Disease Consortium.
• According to local protocols, patients were treated.
• Before and during treatment, researchers measured annualized relapse rates (ARRs) and Expanded Disability Status Scale (EDSS) scores with DMDs.

Results

• Researchers identified 102 children (median [range] age, 7.0 [1.5-7.9] years; male to female ratio, 1.0:1.8; white to other race/ethnicity ratio, 3.6:1.0).
• in this study, original diagnoses were neuromyelitis optica spectrum disorder (44 patients [43.1%]), acute disseminated encephalomyelitis followed by optic neuritis (20 [19.6%]), multiphasic disseminated encephalomyelitis (20 [19.6%]), and relapsing optic neuritis (18 [17.6%]).
• In all, there were reports of 464 demyelinating events.
• Treated patients, compared to untreated patients, had more relapses (median, 3.0 vs 1.0; range, 1.0-17.0 vs 1.0-7.0) (P=.009) and higher EDSS scores (median, 1.5 vs 1.0; interquartile range, 0-2.5 vs 0-1.5) (P < .001).
• DMDs were administered to 52 children (51.0%): 28 (53.8%) were treated with 1 DMD, 17 (32.7%) with 2, and 7 (13.5%) with 3 or more sequential DMDs.
• During all treatments, patients relapsed; there were reports of 127 relapses on treatment.
• Between the preinitiation and postinitiation phases of interferon beta and glatiramer acetate treatment, no changes in median ARR and EDSS score were noticed (n = 11).
• Although the median EDSS score remained unchanged, the median ARR was reduced from 1.84 to 1.0 with azathioprine (n = 20, P < .001), 1.79 to 0.52 with mycophenolate mofetil (n = 15, P=.003), and 2.12 to 0.67 with rituximab (n = 9, P < .001).
• Twelve patients treated with regular intravenous immunoglobulins showed an improvement in ARR (from 2.16 to 0.51, P < .001) and EDSS score (from 2.2 to 1.2, P=.01).