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Abstract Details

A Review of Pediatric Demyelinating Disorders to Determine the Clinical Presentation and Imaging Spectrum of Myelin Oligodendrocyte Glycoprotein Antibody Disease
Autoimmune Neurology
Autoimmune Neurology Posters (7:00 AM-5:00 PM)
056
To identify features of myelin oligodendrocyte glycoprotein (MOG) antibody cases in children who present with pediatric demyelinating disorders to improve its recognition and differentiation from similar demyelinating disorders.

The myelin oligodendrocyte protein is expressed on myelin sheaths of oligodendrocytes. The exact biology of MOG antibody remains unclear, but animal studies show it is an encephalitogenic protein. Myelin oligodendrocyte antibody disease is a newly recognized demyelinating disorder that has not been fully elucidated. It is possible MOG-antibody associated encephalomyelitis is a separate demyelinating entity with overlapping features of other pediatric demyelinating disorders: acute disseminated encephalomyelitis, neuromyelitis optica spectrum disorder, and multiple sclerosis.

A retrospective review was conducted of all pediatric patients presenting with demyelinating disorder to a specialized pediatric institution. Of the 270 total cases, 16 cases with lab confirmed MOG antibody disease were identified. Electronic medical records were reviewed for patient demographics, clinical characteristics, labs, and disease course including relapses. Imaging was reviewed by a pediatric neuroradiologist.

The mean (SD) patient age was 12.5 (4.7) years; age at disease onset was 8.6 (4.8) years, and number of relapses was 1.1 (1.6). Patient demographics: 25% male, nine (56.3%) White patients, six (37.5%) Hispanic patients, and one (6.25%) African American patient. Three patients tested positive for oligoclonal bands, one had an elevated IgG index, two had elevated IgG synthesis rates, and all were negative for AQP4. Fever was noted in 43.8% of presentations. No associated autoimmune disorders. Spine MRI showed central longitudinally extensive transverse myelitis and conus involvement. Brain MRI findings were more often bilateral than unilateral, lesions in the brachium pontis and subcortical white matter were larger than typical MS lesions, and midbrain abnormalities often involved periaqueductal gray matter.

These clinical findings aid in differentiating MOG antibody from similar neurological conditions and support further investigation of this disease in pediatric patients.
Authors/Disclosures
Jeffrey Varghese
PRESENTER
Mr. Varghese has nothing to disclose.
No disclosure on file
Jennifer P. Rubin, MD (Ann & Robert H Lurie Children's Hospital of Chicago) Dr. Rubin has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Passage Bio.
No disclosure on file