Immunobiology of neuromyelitis optica spectrum disorders

Abstract

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune inflammatory disease of the central nervous system. Most of the cases are positive for autoantibodies targeting the water channel aquaporin-4 (AQP4-IgG). Activated B and T cells, innate immunity cells, pro-inflammatory cytokines, and activated complement contribute to the formation of the NMOSD lesions. Optic neuritis, longitudinally extensive myelitis, and area postrema syndrome are core clinical manifestations. NMOSD diagnosis is based on clinical manifestations, magnetic resonance imaging findings, and AQP4-IgG positivity. Cell-based assays are the preferred method for the detection of AQP4-IgG. Acute relapses are treated with IV methylprednisolone or plasma exchange. Recent advances on the NMOSD immunobiology led to approved treatments such as eculizumab, satralizumab, and inebilizumab.