Abstract
•Experts in Saudi Arabia have developed comprehensive guidelines on the diagnosis and management of Neuromyelitis Optica spectrum disorder.•Treatment with Immunosuppressants such as rituximab requires monitoring of CD19 levels, organ functions, and latent infections.•Replacement with intravenous immunoglobulins is recommended for patients with a serum total IgG level below 150.•Methotrexate, Mycophenolate mofetil, and azathioprine are all acceptable treatment medications with rituximab being administered as a second-line agent.•All patients positive for aquaporin-4 antibodies should receive long-term immunosuppression with a steroid-sparing medication.
This article focuses on the diagnosis and management of neuromyelitis optica spectrum disorder (NMOSD). NMOSD is an autoimmune, demyelinating condition characterized by inflammation of the optic nerve and/or the spinal cord, with symptoms that can range from mild impairment of movement to paralysis. The newly approved diagnostic criteria have improved the accuracy of NMOSD diagnosis. The management of NMOSD is under major revolution due to the many new therapeutic options. The role of the antibodies directed at aquaporin-4 (AQP4) has materialized as a biomarker for NMOSD. Several new treatments that target variable aspects in immunopathology such as IL-6, complement, or depletion of B cells are emerging. The management of AQP4-negative patients remains challenging.