TY - JOUR T1 - Neuromyelitis optica spectrum disorders JF - Clinical Medicine JO - Clin Med SP - 169 LP - 176 DO - 10.7861/clinmedicine.19-2-169 VL - 19 IS - 2 AU - Saif Huda AU - Dan Whittam AU - Maneesh Bhojak AU - Jayne Chamberlain AU - Carmel Noonan AU - Anu Jacob AU - Rachel Kneen Y1 - 2019/03/01 UR - http://www.rcpjournals.org/content/19/2/169.abstract N2 - Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon antibody-mediated disease of the central nervous system. Long segments of spinal cord inflammation (myelitis), severe optic neuritis, and/or bouts of intractable vomiting and hiccoughs (area postrema syndrome) are classic presentations of the disease and may alert the clinician to the diagnosis. Untreated, approximately 50% of NMOSD patients will be wheelchair users and blind, and a third will have died within 5 years of their first attack. Unlike multiple sclerosis, a progressive clinical course is very unusual and the accrual of disability is related to relapses. Approximately 75% of patients have antibodies against aquaporin-4, a water channel expressed on astrocytes. Relapses are treated aggressively to prevent residual disability with high-dose steroids and often plasma exchange. Relapse prevention is crucial and achieved with long-term immunosuppression. In this article we review the pathogenesis, clinical features, diagnosis and management of NMOSD. ER -