Author(s): Huda S.; Whittam D.; Bhojak M.; Noonan C.; Jacob A.; Chamberlain J.

Source: Clinical medicine (London, England); Mar 2019; vol. 19 (no. 2); p. 169-176

Publication Date: Mar 2019

Publication Type(s): Review

PubMedID: 30872305

Available  at Clinical medicine (London, England) –  from ProQuest (Hospital Premium Collection) – NHS Version

Available  at Clinical medicine (London, England) –  from EBSCO (MEDLINE Complete)

Available  at Clinical medicine (London, England) –  from Edge Hill Aintree LIRC (lib302411) Local Print Collection [location] : Edge Hill Aintree LIRC.

Abstract: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.

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

Database: EMBASE

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