Moheb, Negar

Neuromyelitis optica spectrum disorder (NMOSD) is a severe autoimmune disease that predominantly affects the optic nerves and spinal cord, which is a distinct disease process from multiple sclerosis (MS) and myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease. The most common neuro-ophthalmologic manifestation of NMOSD is atypical optic neuritis (ON), characterized by severe vision loss with a higher propensity toward bilateral involvement, longitudinal enhancement of the optic nerve, and chiasmal involvement than MS-related optic neuritis. Due to the severity of disease, high recurrence rate, and significant risk of permanent deficits, early recognition of neuro-ophthalmologic signs and short- and long-term management with immunosuppressive therapies is critical. In this article, we will review the characteristic neuro-ophthalmologic findings in NMOSD, serologic markers, neuroimaging findings, and current approaches to both acute and long-term treatment.