Neuromyelitis optica: clinical syndrome and the NMO-IgG autoantibody marker.
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.
Weinshenker
B G
BG
Department of Neurology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55901, USA. weinb@mayo.edu
Wingerchuk
D M
DM
D000818:Animals; D001323:Autoantibodies / Q000097:blood*; D015415:Biomarkers / Q000097:blood*; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood* / Q000276:immunology; D051379:Mice; D009471:Neuromyelitis Optica / Q000276:immunology / Q000503:physiopathology*
D001323:Autoantibodies; D015415:Biomarkers; D007074:Immunoglobulin G