WCN 2021 | Diagnostic criteria and treatment updates for NMOSD

Kazuo Fujihara, MD, PhD, Fukushima Medical University School of Medicine, Fukushima, Japan, outlines the revised diagnostic criteria for neuromyelitis optica spectrum disorder (NMOSD) and discusses advances in the treatment of this condition. NMOSD is an inflammatory central nervous system (CNS) syndrome characterized by severe optic neuritis and longitudinally extensive transverse myelitis lesions. Following the discovery that many patients had detectable serum antibodies targeting aquaporin-4 (AQP4), the diagnostic criteria for NMOSD were revised in 2015 and stratified the disease based on aquaporin-4 immunoglobulin G antibodies (AQP4-IgG) serostatus. Importantly, these new criteria facilitated the diagnosis of AQP4-IgG-positive NMOSD as it stated that only one core clinical characteristic from optic neuritis, acute myelitis, and brain syndrome was necessary for a diagnosis. Further research must be conducted to improve our understanding of seronegative NMOSD, which is considerably more heterogeneous. Moreover, three practice-changing randomized controlled trials evaluating the monoclonal antibodies eculizumab (NCT01892345), inebilizumab (N-MOmentum, NCT02200770), and satralizumab (NCT02028884) showed a remarkable reduction in the risk of relapse ranging from 80-90% and reported a tolerable safety profile. Dr Fujihara explains that in the future, the challenge will be to find effective treatments for seronegative NMOSD. This interview took place during the XXV World Congress of Neurology.