title: Neuromyelitis optica: clinical features, immunopathogenesis and treatment.
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.
authors.affiliation: Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Jarius
authors.forename: B
authors.initials: B
authors.lastname: Wildemann
authors.forename: F
authors.initials: F
authors.lastname: Paul
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D051401:Aquaporin 4 / Q000276:immunology; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D009471:Neuromyelitis Optica / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology*; D011379:Prognosis
chemical_list: D000911:Antibodies, Monoclonal; D051401:Aquaporin 4; D001323:Autoantibodies; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents
keywords: Devic syndrome; NMO-IgG; aquaporin-4 antibodies (AQP4); diagnosis; neuromyelitis optica; pathogenesis; pathophysiology; treatment