• Saif Huda, Dan Whittam, Maneesh Bhojak, Jayne Chamberlain, Carmel Noonan, and Anu Jacob.
• Walton Centre NHS Foundation Trust, Liverpool, UK shuda@nhs.net.
• Clin Med (Lond). 2019 Mar 1; 19 (2): 169176169-176.

AbstractNeuromyelitis 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.© Royal College of Physicians 2019. All rights reserved.

19 keywords...

hide…