[Recent advances in the treatment of neuromyelitis optica]

Neuromyelitis optica is an autoimmune-mediated inflammatory disease affected predominantly optic nerve and spinal cord. The pathogenic mechanism was found to be distinct from multiple sclerosis since the discovery of the disease specific autoantibody against water channel aquaporin-4. The pathogenicity of anti-aquporin-4 antibody causes complement-dependent and cell-mediated astrocyte damage in the central nervous system, resulting in the severe inflammatory lesions including loss of aquaporin-4. The recent elucidation of pathological mechanism contributed to the development of treatment with monoclonal antibodies, e.g., rituximab, tocilizumab and eculizumab. It also raises the possibility that neutralization of the pathogenic autoantibodies, attenuation of polymorphonuclear leukocyte accumulation, and regulation of the proinflammatory cytokine and chemokine production can be efficacious for acute exacerbation and prevention of the relapses.