Introduction: Neuromyelitis optica spectrum disorders (NMOSD) are autoimmune diseases of the central nervous system mainly involving the optic nerves and spinal cord. Many advances have been made in understanding the immunopathology of NMOSD and related clinical classification, nevertheless, open issues in management and effective therapeutic approaches still remain. Areas covered: In this article, the authors reviewed and discussed the scientific evidence in pathogenesis and pharmacological therapy of NMOSD addressing the more recent advances in new biological treatment option and therapeutic strategy that may help to improve management of this condition. Expert opinion: Despite current immunopathogenic evidence, NMOSD management represents a challenge due to the poor-validated diagnostic, prognostic and therapeutic biomarkers. A tailored approach is mandatory to improve the management of the different disease clinical settings.
Keywords: Neuromyelitis optica spectrum disorders (NMOSD); aquaporin-4 immunoglobulin G (AQP4-IgG); diagnostic criteria; emerging treatment; immunopathogenesis; management; myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG); standard treatment.