Differential diagnosis of neuromyelitis optica spectrum disorders – is it easy to make a mistake? Review article

Abstract

Neuromyelitis optica spectrum disorders (NMOSD) is an autoimmune disease of the central nervous system that primarily affects the optic nerves and the spinal cord. Formerly this disease entity was considered to be a variant of multiple sclerosis (MS), but now we can distinguish these diseases in terms of clinical, pathomorphological and immunological. A breakthrough in the diagnosis of NMOSD was in 2004 the identification of antibodies against aquaporin 4 (AQP4-IgG). This helped us to understand the essence of the disease and its serological diagnostics. Although it soon turned out that AQP4-IgG were not present in all cases but their detection gives the possibility of rapid diagnosis in about 75% of patients. The detection of myelin oligodendrocyte glycoprotein (anti-MOG) antibodies in some patients completed the NMOSD picture. Differentiation between NMOSD and MS is extremely important due to different prognosis and treatment. Diagnosis of NMOSD was associated with very poor prognosis and the available immunosuppressive therapies were insufficiently effective. The improvement in diagnostic methods goes hand in hand with the emergence of new therapies that will hopefully change the unfavourable course of NMOSD.