NEURO-OPHTHALMOLOGY: Edited by Dean M. CestariUpdate on neuromyelitis optica spectrum disorderHolroyd, Kathryn B.a,∗; Manzano, Giovanna S.a,∗; Levy, MichaelbAuthor Information aHarvard Mass General Brigham Neurology Residency bDepartment of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA Correspondence to Michael Levy, MD, PhD, Associate Professor of Neurology, Harvard Medical School; Research Director, Division of Neuroimmunology and Neuroinfectious Disease, Massachusetts General Hospital, 55 Fruit St., Boston, MA 02114, USA. E-mail: email@example.com Current Opinion in Ophthalmology: November 2020 - Volume 31 - Issue 6 - p 462-468 doi: 10.1097/ICU.0000000000000703 Buy Metrics Abstract Purpose of review Neuromyelitis optica spectrum disorder is an autoimmune disease that causes optic neuritis and transverse myelitis. Attacks can cause severe neurological damage leading to blindness and paralysis. Understanding of the immunopathogenesis of this disease has led to major breakthroughs in diagnosis and treatment. In the past 18 months, three successful phase 3 clinical trials have been published using targeted approaches to preventing relapses. Recent findings Updates in epidemiology, imaging, quality of life and treatment for acute relapse and prevention have been published in the past 18 months. Epidemiology studies are distinguishing patients based on their antigen specificity for aquaporin-4 and myelin oligodendrocyte glycoprotein, which are increasingly recognized as separate immunological conditions. Imaging by MRI and optical coherence tomography continue to be developed as tools to distinguish neuromyelitis optica spectrum disorders (NMOSD) from other diseases. This is especially relevant as the recent clinical trials showed differences in response between aquaporin-4 seropositive and seronegative patients. The three drugs that were tested for prevention of NMOSD relapses were eculizumab, inebilizumab, and satralizumab. All of the trials were worldwide, placebo-controlled, double-masked studies that demonstrated a clear benefit with each approach. Summary Recent research in NMOSD has resulted in improved diagnosis and approved treatments. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.