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Diagnosis and management of neuromyelitis optica spectrum disorders - An update
Authors:Alice Bruscolini  Marta Sacchetti  Maurizio La Cava  Magda Gharbiya  Massimo Ralli  Alessandro Lambiase  Armando De Virgilio  Antonio Greco
Affiliation:1. Department of Sense Organs, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy;2. Department of Oral and Maxillofacial Sciences, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy;3. Otorhinolaryngology Unit, Humanitas Clinical and Research Center, Via Alessandro Manzoni, 56, 20089 Rozzano (MI), Italy
Abstract:
Neuromyelitis optica (NMO) and Neuromyelitis optica spectrum disorders (NMOSD) are a group of autoimmune conditions characterized by inflammatory involvement of the optic nerve, spinal cord and central nervous system. Novel evidence showed a key role of autoantibodies against aquaporin-4 immunoglobulin G (AQP4 IgG) in the pathogenesis of NMOSD and, recently, new classification and diagnostic criteria have been adopted to facilitate an earlier identification and improve the management of these conditions. Diagnosis of NMOSD is currently based on clinical, neuroimaging and laboratory features. Standard treatment is based on the use of steroids and immunosuppressive drugs and aims to control the severity of acute attacks and to prevent relapses of the disease. This review gives an update of latest knowledge of NMOSD and NMO, emphasizing the novel diagnostic criteria and both current and future therapeutic approaches.
Keywords:AQP4  aquaporin-4  AQP4 IgG  immunoglobulin G against aquaporin-4  ARR  Annualized Relapse Rates  AZT  azathioprine  BBB  blood-brain barrier  CNS  central nervous system  EDSS  Expanded Disability Status Scale  GFAP  glial fibrillary acid protein  ICAM1  intercellular adhesion molecule-1  LETM  longitudinally extensive transverse myelitis  MM  mycophenolate mofetil  MOG  myelin oligodendrocytes glycoprotein  MS  multiple sclerosis  NMO  neuromyelitis optica  NMOSD  neuromyelitis optica spectrum disorders  OCT  ocular coherence tomography  ON  optic neuritis  PLEX  plasma exchange  PNS  peripheral nervous system  VEP  visual evoked potential  VCAM1  vascular adhesion molecule-1  VEGF A  endothelial growth factor-A  Neuromyelitis optica  Neuromyelitis optica spectrum disorders  Aquaporin-4 immunoglobulin G  Diagnostic criteria  Management
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