Comparisons of clinical phenotype, radiological and laboratory features, and therapy of neuromyelitis optica spectrum disorder by regions: update and challenges

Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory demyelinating disease of the central nervous system (CNS) associated with autoantibody (ab) to aquaporin-4 (AQP4). There is obvious variation between regions and countries in the epidemiology, clinical features and management in NMOSD. Based on published population-based observation and cohort studies, the different clinical pattern of NMOSD has been seen in several geographical regions and some of these patients with NMOSD-like features do not fully meet the current diagnostic criteria, which is needed to consider the value of recently revised diagnostic criteria. At present, all treatments applied in NMOSD have made great progress, however, these treatments failed in AQP4 ab negative and refractory patients. Therefore, it is necessary to turn into an innovative idea and to open a new era of NMOSD treatment to develop novel and diverse targets and effective therapeutic drugs in NMOSD and to conduct the trails in large clinical samples and case-control studies to confirm their therapeutic effects on NMOSD in the future, which still remain a challenge.

Automated summary

Neuromyelitis optica spectrum disorder (NMOSD) shows significant variation in epidemiology, clinical features, and management between regions and countries. Current diagnostic criteria need to be reevaluated based on the different clinical patterns observed in various geographical regions. Although progress has been made in treatment, novel therapeutic targets and drugs are needed, especially for patients who are negative for aquaporin-4 antibody and refractory to current treatments.