Pediatric Neuromyelitis Optica Spectrum Disorder

Neuromyelitis Optica Spectrum Disorder (NMOSD) is a demyelinating disease with a high relapse rate and risk of disability accrual. The condition is an astrocytopathy, with antibod-ies to the aquaporin-4 (AQP4) water channel being detected in AQP4-IgG seropositive dis-ease. Presentation is uncommon in the pediatric age range, accounting for about 3%-5% of cases. NMOSD is more prevalent in populations of Black or East Asian ancestry. Core clini-cal syndromes include optic neuritis, acute myelitis, area postrema syndrome, acute brain -stem syndrome, acute diencephalic syndrome, and symptomatic cerebral syndrome. First -line treatment options in pediatrics include rituximab, azathioprine, and mycophenolate mofetil. Over half of children with AQP4-IgG seropositive NMOSD develop permanent dis-ability, particularly in visual and motor domains. Novel therapeutic targets in the adult popu-lation have been developed and are changing the treatment landscape for this disorder. Semin Pediatr Neurol 46:101051 & COPY; 2023 Elsevier Inc. All rights reserved.