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Neuromyelitis optica: clinical features, immunopathogenesis and treatment

Item Type:Review
Title:Neuromyelitis optica: clinical features, immunopathogenesis and treatment
Creators Name:Jarius, S. and Wildemann, B. and Paul, F.
Abstract:The term 'neuromyelitis optica' ('Devic's syndrome', NMO) refers to a syndrome characterized by optic neuritis and myelitis. In recent years, the condition has raised enormous interest among scientists and clinical neurologists, fuelled by the detection of a specific serum immunoglobulin (Ig)G reactivity (NMO-IgG) in up to 80% of patients with NMO. These autoantibodies were later shown to target aquaporin-4 (AQP4), the most abundant water channel in the central nervous system (CNS). Here we give an up-to-date overview of the clinical and paraclinical features, immunopathogenesis and treatment of NMO. We discuss the widening clinical spectrum of AQP4-related autoimmunity, the role of magnetic resonance imaging (MRI) and new diagnostic means such as optical coherence tomography in the diagnosis of NMO, the role of NMO-IgG, T cells and granulocytes in the pathophysiology of NMO, and outline prospects for new and emerging therapies for this rare, but often devastating condition.
Keywords:Aquaporin-4 Antibodies (AQP4), Devic Syndrome, Ddiagnosis, Neuromyelitis Optica, NMO-IgG, Pathogenesis, Pathophysiology, Treatment
Source:Clinical and Experimental Immunology
ISSN:0009-9104
Publisher:Wiley-Blackwell (U.K.)
Volume:176
Number:2
Page Range:149-164
Date:May 2014
Official Publication:https://doi.org/10.1111/cei.12271
PubMed:View item in PubMed

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