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Related Experiment Videos

Neuromyelitis optica.

D M Wingerchuk1

  • 1Multiple Sclerosis Center/Department of Neurology, Mayo Clinic College of Medicine, Scottsdale, AZ 85259, USA. wingerchuk.dean@mayo.edu

International MS Journal
|April 26, 2006
PubMed
Summary
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Neuromyelitis optica (NMO), or Devic's disease, is an autoimmune disorder targeting the optic nerves and spinal cord. Early immunosuppression is crucial for managing this severe condition and preventing disability.

Area of Science:

  • Neuroimmunology
  • Autoimmune diseases
  • Inflammatory disorders

Background:

  • Neuromyelitis optica (NMO), also known as Devic's disease, is a severe inflammatory condition.
  • NMO primarily affects the optic nerves and spinal cord.
  • Evidence suggests NMO is a distinct humorally mediated disease, separate from Multiple Sclerosis (MS).

Purpose of the Study:

  • To differentiate Neuromyelitis Optica (NMO) from Multiple Sclerosis (MS).
  • To identify diagnostic criteria and biomarkers for NMO.
  • To understand the immunopathology and prognosis of NMO.

Main Methods:

  • Analysis of clinical, laboratory, and immunopathological data.
  • Evaluation of spinal cord lesion characteristics (longitudinally extensive transverse myelitis).

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  • Assessment of cranial magnetic resonance imaging (MRI) findings in conjunction with clinical presentation.
  • Detection of serum autoantibodies, specifically neuromyelitis optica-immunoglobulin G (NMO-IgG).

    • Main Results:

    • A spinal cord lesion spanning three or more vertebral segments, combined with MRI findings inconsistent with MS, shows high sensitivity (>94%) and specificity (>96%) for NMO diagnosis.
    • The presence of NMO-IgG autoantibodies is specific to NMO.
    • NMO-IgG targets aquaporin-4, a water channel, suggesting NMO as a potential autoimmune channelopathy.
    • The disease spectrum may include recurrent longitudinally extensive transverse myelitis and Japanese opticospinal MS.

    Conclusions:

    • Neuromyelitis optica (NMO) is a distinct autoimmune channelopathy targeting aquaporin-4.
    • Diagnostic criteria involving spinal cord lesions and specific MRI findings enhance NMO diagnosis.
    • Early immunosuppressive therapy is vital for managing relapsing NMO and mitigating disability due to its poor prognosis.