title: Treatment of neuromyelitis optica.
abstract: OBJECTIVE
  Neuromyelitis optica (NMO) is an antibody-mediated inflammatory disease of the central nervous system with a predilection for the optic nerves, spinal cord and certain brain regions. It has a distinct pathogenesis relating to aquaporin-4 autoimmunity and complement-mediated injury. This knowledge has translated into targeted efforts to develop novel, disease-specific treatments. In this review, we discuss evidence supporting the use of currently available treatments for acute exacerbations and for long-term disease modification. We also discuss the risks and benefits of available and emerging immunotherapies.
  
  
  RESULTS
  Early, accurate diagnosis of NMO with appropriate acute and long-term immunosuppressive treatment is of prime importance for the prevention of disability associated with this disease. Standard measures for the management of acute exacerbations include intravenous methylprednisolone and plasmapheresis. First-line, long-term immunotherapies for NMO include azathioprine, mycophenolate mofetil and rituximab. Three randomized controlled treatment trials evaluating these agents are currently being conducted. In addition, there are numerous emerging therapies that are based upon current understanding of the disease immunopathogenesis.
  
  
  CONCLUSIONS
  NMO is an autoimmune disease that is separate from multiple sclerosis. Better understanding of its antibody and complement-dependent pathophysiology has proven to be critical for the formulation of current and future treatment strategies.
authors.affiliation: aHarvard Medical School bMassachusetts General Hospital, Department of Neurology cBrigham and Women's Hospital, Department of Neurology, Boston, Massachusetts, USA.
authors.forename: Ivana
authors.initials: I
authors.lastname: Vodopivec
authors.forename: Marcelo
authors.initials: M
authors.lastname: Matiello
authors.forename: Sashank
authors.initials: S
authors.lastname: Prasad
mesh_terms: D000818:Animals; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009173:Mycophenolic Acid / Q000031:analogs & derivatives; D009471:Neuromyelitis Optica; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D000069283:Rituximab; D009173:Mycophenolic Acid