Neuromyelitis optica: clinical features, immunopathogenesis and treatment. (9th April 2014)

• Record Type: Journal Article
• Authors: Jarius, S; Wildemann, B; Paul, F
• Is Part Of: Clinical and experimental immunology. Volume 176:Number 2(2014:May)
• Page Start: 149
• Page End: 164
• Publication Date: 2014-04-09
• Subjects: aquaporin-4 antibodies (AQP4) -- Devic syndrome -- diagnosis -- neuromyelitis optica -- NMO-IgG -- pathogenesis -- pathophysiology -- treatment; Immunopathology -- Periodicals; 616.079
• DOI: 10.1111/cei.12271
• Languages: English
• ISSNs: 0009-9104

Abstract

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.