The Clinical Spectrum of Myelin Oligodendrocyte Glycoprotein Antibody-associated Disease: A Single Center Experience
Eva Chava Bernfeld1, Lama Abdel Wahed2, Tracey Cho2
1University of Iowa, Pediatric Neurology, 2University of Iowa, Neurology
Objective:
We aim to review Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD) cases at our institution, to complement existing published data for future changes to disease guidelines.
Background:
Most published literature on MOGAD focuses on typical rather than atypical MOGAD presentations. Published MOGAD guidelines emphasize the need for further studies for validation.
Design/Methods:
This is a descriptive retrospective case series. Positive serum anti-Myelin Oligodendrocytes Glycoprotein (MOG) antibody titers between March 2018 to June of 2023 were identified through EHR reporting tools and charts were accessed for review on or before September 18, 2023. IRB approval was obtained.
Results:
Seventy-two patients had positive anti-MOG antibody titers and fifty-five patients met criteria for MOGAD. Of those, 65.5% were female and 32.7% were children. Most commonly, MOGAD presented as optic neuritis in 74.5%, acute disseminated encephalomyelitis (ADEM) in 23.6%, and transverse myelitis in 14.5% of cases. Atypical syndromes included myeloradiculitis, seizures, concomitant NMDA positivity, crossover tumefactive demyelinating syndrome, isolated brainstem involvement, adult ADEM and transverse myelitis in pregnancy. Disease recurred in 43.6% patients overall (44.4% of pediatric and 43.2% of adult patients). Serum anti-MOG antibody titers ranged between 1:20-1:10000. Upon presentation, most patients received intravenous steroids, with some receiving additional IVIG or PLEX. 63.6% of patients (50% of pediatric and 70.2% of adult patients) received maintenance therapy, most commonly rituximab followed by mycophenolate mofetil in adults and IVIG in children.
Conclusions:
In addition to typical MOGAD phenotypes, we describe atypical cases to complement existing data on the broader clinical spectrum of MOGAD. We found a higher MOGAD recurrence rate in our pediatric sub-cohort compared to our adult sub-cohort. Additional longitudinal studies are needed to further characterize the disease characteristics and guide management.