Neuromyelitis optica: Clinical syndrome and the NMO-IgG autoantibody marker

B. G. Weinshenker, D. M. Wingerchuk

Research output: Chapter in Book/Report/Conference proceedingChapter

35 Scopus citations

Abstract

Neuromyelitis optica (NMO) is a severe demyelinating disease of the CNS that preferentially affects the optic nerves and spinal cord, tends to relapse, and results in early permanent disability for most affected patients. A new autoantibody marker called neuromyelitis optica immunoglobulin G (NMO-IgG), which targets the water channel protein aquaporin-4, is highly specific for NMO. The marker has demonstrated that the NMO spectrum of disorders is wider than previously known and includes some patients with single-episode or recurrent longitudinally extensive myelitis, recurrent isolated optic neuritis, Asian optic-spinal multiple sclerosis, and patients with co-existing systemic autoimmune diseases such as lupus erythematosus or Sjögren's syndrome. We review the place of NMO within the nosology of CNS demyelinating diseases, the discovery of NMO-IgG and its impact on the definition of NMO and its spectrum, implications for understanding NMO pathogenesis, and informing treatment decisions.

Original languageEnglish (US)
Title of host publicationAdvances in multiple Sclerosis and Experimental Demyelinating Diseases
EditorsMoses Rodriguez
Pages343-356
Number of pages14
DOIs
StatePublished - Dec 1 2008

Publication series

NameCurrent Topics in Microbiology and Immunology
Volume318
ISSN (Print)0070-217X

ASJC Scopus subject areas

  • Immunology and Allergy
  • Microbiology
  • Immunology
  • Microbiology (medical)

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