Immunobiology of neuromyelitis optica spectrum disorders. (October 2022)

• Record Type: Journal Article
• Authors: Mora Cuervo, Daissy Liliana; Hansel, Gisele; Sato, Douglas Kazutoshi
• Is Part Of: Current opinion in neurobiology. Volume 76(2022)
• Publication Date: 2022-10
• Subjects: Neurobiology -- Periodicals; 573.8
• DOI: 10.1016/j.conb.2022.102618
• Languages: English
• ISSNs: 0959-4388

Abstract

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 . HIGHLIGHTS: Autoimmunity against central nervous system is a key factor in the neuromyelitis optica spectrum disorder (NMOSD) pathogenesis. Astrocytes expressing aquaporin-4 are the main target to aquaporin-4 autoantibodies in NMOSD. Activated immune cells, cytokines, and activated complement participate in the formation of NMOSD lesions. Approved treatments for NMOSD (Eculizumab, Satralizumab, Inebilizumab) are highly effective in the reduction of NMOSD attacks.