Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune, inflammatory 
disorder of the central nervous system that typically presents with recurrent 
episodes of optic neuritis, longitudinally extensive myelitis, brainstem, 
diencephalic, and cerebral syndromes. Up to 80% of NMOSD patients have a 
circulating pathogenic autoantibody that targets the water channel aquaporin-4 
(AQP4-IgG). The discovery of AQP4-IgG transformed our understanding of the 
pathogenesis of the disease and its possible treatment targets. Monoclonal 
antibodies targeting terminal complement (eculizumab), CD19 (inebilizumab), and 
the interleukin-6 receptor (satralizumab) have demonstrated efficacy in NMOSD 
attack prevention in recent phase 3 trials and have gained subsequent regulatory 
approval in the USA and other countries. We aim to review the evidence 
supporting the efficacy of these new drugs.