Advances in research on neuromyelitis optica spectrum disorders and its clinical heterogeneity

Abstract

Neuromyelitis optica spectrum disorders (NMOSD) is a demyelinating disease mainly involving the optic nerve and spinal cord. It has recurrent and aggravating attacks and high disability rate. Most patients have a stepwise progression, resulting in complete blindness or paraplegia. NMOSD lesions contain not only the optic nerve and spinal cord, but also other neurological and non-neurological symptoms, which has clinical heterogeneity. The discovery of aquaporin-4-immunoglobulin G (AQP4-IgG) attributed it to autoimmune ion-channel disease, and rituximab (RTX) has achieved good clinical efficacy in the treatment of NMOSD. Myelin oligodendrocyte glycoprotein (MOG) antibodies have been found in some AQP4-IgG-negative NMOSD patients, which have different clinical and immunological features, posing new challenges to the diagnosis and treatment of NMOSD, which may require re-design and testing of new immune-targeted drugs.

Keywords

neuromyelitis optica spectrum disorders (NMOSD) / aquaporin-4-immunoglobulin G (AQP4-IgG) / myelin oligodendrocyte glycoprotein (MOG) / rituximab (RTX) / paraneoplastic neurological syndrome