To describe the clinical course and treatment response of patients with myelin oligodendrocyte glycoprotein antibody (MOG-IgG) disease at a freestanding children’s hospital. 

Although pediatric MOG-IgG disease is increasingly well-recognized, the full clinical spectrum of the disease is still being defined.

A retrospective case series was performed using review of electronic medical record from July 2018 to September 2019.

Twenty pediatric patients who presented with suspected central nervous system (CNS) demyelinating/inflammatory diseases were tested for serum MOG-IgG titers using a cell-based assay. Six (30%) of the 20 patients were found to have positive serum MOG-IgG. Three out of the six patients have had monophasic courses: two with acute disseminated encephalomyelitis (ADEM), and one with bilateral optic neuritis (ON). The other three patients have had relapsing courses: one with ADEM followed by bilateral ON, one with four attacks of recurrent meningoencephalitis, and one with two attacks of ADEM and one attack of isolated ON. Two patients with relapsing disease were treated with rituximab without further relapses in 12 months of follow-up. The third patient relapsed while receiving monthly intravenous immunoglobulin; mycophenolate mofetil was then added without subsequent relapses in five months of follow-up. Two patients had elevated intracranial pressure during acute attacks, one of which had undergone ventriculoperitoneal shunt placement prior to receiving MOG-IgG testing. Three patients were tested for respiratory viruses, including influenza A/B, during their initial attacks, and one patient with monophasic ADEM tested positive for adenovirus.

Thirty percent of pediatric patients who presented with suspected CNS demyelinating/inflammatory disease were found to have positive serum MOG-IgG titers. Three of six patients with MOG-IgG disease have had at least one relapse. Two patients with relapsing disease have had elevated opening pressures during acute attacks, suggesting increased intracranial pressure may be associated with MOG-IgG disease.