Neuromyelitis optica spectrum disorders are rare autoimmune inflammatory disorders of the central nervous system predominantly targeting the optic nerves and spinal cord, but also the brain and brainstem. The discovery of aquaporin-4 immunoglobulin G (AQP4-IgG) has led to increased understanding of pathogenesis and a diverse spectrum of disorders and has improved the diagnostics of this disease.
Typical disease course is relapsing-remitting. During a relapse, new inflammatory burden can lead to accumulating disability.
The results of four different clinical trials have recently been published. Three monoclonal antibodies (eculizumab, satralizumab and inebilizumab) have shown effect on reduction in risk of relapse.