Neuromyelitis optica spectrum disorder (NMOSD) is an infrequent inflammatory autoimmune disorder of the central nervous system, that affects the spinal cord and optic nerves. Aquaporin 4 IgG (AQP4-IgG) is a seropositive-specific antibody detected in the serum of a patient with NMO. AQP4-IgG binds to AQP4 via the astrocyte end-feet, causing a classical complement cascade that results in an inflammatory response-induced astrocyte injury and a secondary response involving oligodendrocyte loss and demyelination. NMO causes severe relapses and affects women more than men. A patient with NMO experiences pain primarily as transverse myelitis with longitudinally extensive recurrences or bilateral optic neuritis. Treating NMOSD poses considerable clinical challenges. This review provides an overview of the state-of-the-art studies summarizing the pain, prevalence, and primary pathology of NMOSD and discusses several potential therapeutic targets for its treatment.

Keywordsneuromyelitis optica, AQP4-IgG, optic neuritis, multiple sclerosis, rituximab