Neuromyelitis optica: clinical features, immunopathogenesis and treatment

Summary 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 immunoglobuli...

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Bibliographic Details
Published in:Clinical and experimental immunology 2014-05, Vol.176 (2), p.149-164
Main Authors: Jarius, S., Wildemann, B., Paul, F.
Format: Article
Language:English
Subjects:
Antibodies, Monoclonal - therapeutic use
Aquaporin 4 - immunology
aquaporin‐4 antibodies (AQP4)
Autoantibodies - blood
Autoantibodies - immunology
Devic syndrome
diagnosis
Humans
Immunoglobulin G - blood
Immunoglobulin G - immunology
Immunosuppressive Agents - therapeutic use
neuromyelitis optica
Neuromyelitis Optica - diagnosis
Neuromyelitis Optica - drug therapy
Neuromyelitis Optica - immunology
NMO‐IgG
pathogenesis
pathophysiology
Prognosis
Review
treatment
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Summary:Summary 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.
ISSN:0009-9104
1365-2249
DOI:10.1111/cei.12271