Neuromyelitis optica spectrum disorders

Clin Med (Lond). 2019 Mar;19(2):169-176. doi: 10.7861/clinmedicine.19-2-169.


Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon antibody-mediated disease of the central nervous system. Long segments of spinal cord inflammation (myelitis), severe optic neuritis, and/or bouts of intractable vomiting and hiccoughs (area postrema syndrome) are classic presentations of the disease and may alert the clinician to the diagnosis. Untreated, approximately 50% of NMOSD patients will be wheelchair users and blind, and a third will have died within 5 years of their first attack. Unlike multiple sclerosis, a progressive clinical course is very unusual and the accrual of disability is related to relapses. Approximately 75% of patients have antibodies against aquaporin-4, a water channel expressed on astrocytes. Relapses are treated aggressively to prevent residual disability with high-dose steroids and often plasma exchange. Relapse prevention is crucial and achieved with long-term immunosuppression. In this article we review the pathogenesis, clinical features, diagnosis and management of NMOSD.

Keywords: Neuromyelitis optica; antibody; aquaporin-4.

Publication types

  • Review

MeSH terms

  • Adult
  • Aquaporin 4 / immunology
  • Autoantibodies / immunology
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Middle Aged
  • Neuromyelitis Optica* / diagnosis
  • Neuromyelitis Optica* / pathology
  • Neuromyelitis Optica* / physiopathology
  • Neuromyelitis Optica* / therapy
  • Young Adult


  • Aquaporin 4
  • Autoantibodies
  • Immunosuppressive Agents