Optical neuromyelitis spectrum disorders (NMOSD)

Abstract:

Neuromyelitis optica spectrum disorders (NMOSD) is an inflammatory autoimmune disorder of the central nervous system characterized by extensive demyelination and axonal damage that predominantly affects the optic nerves and spinal cord, but involvement of the brainstem and area postrema is also common. NMOSD is characterized by severe disease relapses that may lead to significant neurological impairment, disability and even fatal outcome. The main differential diagnosis of the disease is multiple sclerosis (MS) and the new NMOSD criteria, including clinical features, serological analysis of specific aquaporin IgG antibody (AQP4) as well as magnetic resonance imaging (MR), provide a better understanding of the pathophysiology of the disease and the distinction between these two important demyelination diseases. In recent years, one has witnessed a significant progress in the development of new therapies that target inhibition of complement components and their receptors, which significantly reduce relapse rates and affect long-term patient outcomes.

Key words:
AQP4 antibodies; neuromyelitis optica spectrum; optic neuritis; transverse myelitis