title: Neuromyelitis Optica (Devic's Syndrome): an Appraisal.
abstract: Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD), previously known as Devic's syndrome, are a group of inflammatory disorders of the central nervous system (CNS) characterized by severe, immune-mediated demyelination and axonal damage, predominantly targeting optic nerves and the spinal cord typically associated with a disease-specific serum NMO-IgG antibody that selectively binds aquaporin-4 (AQP4). The classic and best-defined features of NMOSD include acute attacks of bilateral or rapidly sequential optic neuritis (leading to visual loss) or transverse myelitis (often causing limb weakness and bladder dysfunction) or both with a typically relapsing course. The diagnosis of NMO/NMOSD requires a consistent history and examination with typical clinical presentations, findings on spinal cord neuroimaging with MRI, cerebrospinal fluid analysis along with determination of AQP4-IgG serum autoantibody status, and exclusion of other disorders. Two major advances in this field has been the development of diagnostic criteria and treatment recommendations. Consensus diagnostic criteria have been established and were recently revised and published in 2015, enhancing the ability to make a diagnosis and appropriately evaluate these disorders. Expert recommendations and uncontrolled trials form the basis of treatment guidelines. All patients with suspected NMOSD should be treated for acute attacks as soon as possible with high-dose intravenous methylprednisolone -1 gram daily for three to five consecutive days and in some cases, plasma exchange should be used. It is recommended that every patient with NMOSD be started on an immunosuppressive agent, such as, azathioprine, methotrexate, or mycophenolate and in some cases, rituximab, soon after the acute attack and usually be treated for about 5 years after the attack. These advances have helped improve the prognosis and outcome in these disorders.
authors.affiliation: Division of Rheumatology, Department of Medicine, University of Mississippi Medical Center, 2500 N. State Street, Jackson, MS, 39216, USA. tcrout@umc.edu.
authors.forename: Teresa M
authors.initials: TM
authors.lastname: Crout
authors.affiliation: Division of Rheumatology, Department of Medicine, University of Mississippi Medical Center, 2500 N. State Street, Jackson, MS, 39216, USA.
authors.forename: Laura P
authors.initials: LP
authors.lastname: Parks
authors.affiliation: Division of Rheumatology, Department of Medicine, University of Mississippi Medical Center, 2500 N. State Street, Jackson, MS, 39216, USA.
authors.forename: Vikas
authors.initials: V
authors.lastname: Majithia
mesh_terms: D051401:Aquaporin 4 / Q000276:immunology*; D001323:Autoantibodies / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D009471:Neuromyelitis Optica / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000276:immunology*; D011379:Prognosis; D013116:Spinal Cord / Q000000981:diagnostic imaging*
chemical_list: D051401:Aquaporin 4; D001323:Autoantibodies; D007166:Immunosuppressive Agents
keywords: Aquaporin-4 antibody; Central nervous system demyelination; Devic’s syndrome; Diagnosis; Longitudinally extensive transverse myelitis; Neuromyelitis optica; Optic neuritis; Treatment