Neuromyelitis optica: clinical features, immunopathogenesis and treatment

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...

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Hauptverfasser: Jarius, Sven (VerfasserIn) , Wildemann, Brigitte (VerfasserIn) , Paul, Friedemann (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: 13 January 2014
In: Clinical & experimental immunology
Year: 2014, Jahrgang: 176, Heft: 2, Pages: 149-164
ISSN:1365-2249
DOI:10.1111/cei.12271
Online-Zugang:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1111/cei.12271
Verlag, lizenzpflichtig, Volltext: https://onlinelibrary.wiley.com/doi/abs/10.1111/cei.12271
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Verfasserangaben:S. Jarius, B. Wildemann and F. Paul

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520 |a 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. 
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