Up-to-date knowledge of pathogenesis, diagnosis, treatment and care in neuromyelitis optica

Abstract

Neuromyelitis optica spectrum disorders (NMOSD) and multiple sclerosis (MS) are the two main autoimmune disorders of the central nervous system (CNS). The discovery of diagnostic biomarkers for NMOSD, aquaporin–4 (AQP4) autoantibodies, provides significant research progresses of NMOSD as follows ; 1) accurate diagnosis and prompt therapeutic option for patients with NMOSD and MS, 2) newly pathomechanistic insights of AQP4 autoantibodies and complements as significant pathogenic immune molecules for NMOSD, resulting in primary damages of astrocytes and ependymal cells, and secondary but severe neuroaxonal damages and demyelination, and 3) future possible developments of new disease–modifying therapies that target aberrant immune systems (e.g. complements, interleukin–6 and B cells) for NMOSD. The NMOSD landscape has been comprehensively transformed by these progresses since the discovery of AQP4 autoantibodies in 2004.