Neuromyelitis optica: clinical features, immunopathogenesis and treatment

Authors

  • S. Jarius,

    1. Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany
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  • B. Wildemann,

    1. Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany
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  • F. Paul

    Corresponding author
    1. NeuroCure Clinical Research Center and Clinical and Experimental Multiple Sclerosis Research Center, Department of Neurology, Charité University Medicine, Berlin, Germany
    • Correspondence: F. Paul, NeuroCure Clinical Research Center, Charité University Medicine Berlin, Charitéplatz 1, 10117 Berlin, Germany.

      E-mail: friedemann.paul@charite.de

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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.

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