RT Journal Article
SR Electronic
T1 Neuromyelitis optica spectrum disorders
JF Clinical Medicine
JO Clin Med
FD Royal College of Physicians
SP 169
OP 176
DO 10.7861/clinmedicine.19-2-169
VO 19
IS 2
A1 Saif Huda
A1 Dan Whittam
A1 Maneesh Bhojak
A1 Jayne Chamberlain
A1 Carmel Noonan
A1 Anu Jacob
A1 Rachel Kneen
YR 2019
UL http://www.rcpjournals.org/content/19/2/169.abstract
AB 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.