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Neuromyelitis Optica: Clinical Syndrome and the NMO-IgG Autoantibody Marker

  • B. G. Weinshenker
  • D. M. Wingerchuk
Part of the Current Topics in Microbiology and Immunology book series (CT MICROBIOLOGY, volume 318)

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.

Keywords

Multiple Sclerosis Optic Neuritis Systemic Autoimmune Disease Neuromyelitis Optica Inflammatory Demyelinating Disease 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2008

Authors and Affiliations

  • B. G. Weinshenker
    • 1
  • D. M. Wingerchuk
    • 2
  1. 1.Department of NeurologyMayo Clinic College of MedicineRochesterUSA
  2. 2.Department of NeurologyMayo Clinic College of MedicineScottsdaleUSA

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