Double-seronegative neuromyelitis optica: it is not possible to interrupt treatment after 10 years of stability

Neuromyelitis optica spectrum disorder (NMOSD) is a severe autoimmune demyelinating disease characterized by a high risk of relapse and disability. In cases positive for aquaporin 4-immunoglobulin G (AQP4-IgG), long-term immunosuppression is the established standard. However, the management of double-seronegative NMOSD remains controversial due to limited data. Although some authors propose treatment discontinuation in patients with prolonged remission, there are no reliable predictors of sustained disease inactivity. Given the unpredictability and severity of the relapses, even after a decade of stability, and the lack of robust evidence supporting safe cessation, we argue that maintenance immunotherapy should remain the standard of care in most cases. The risks of disease reactivation outweigh the potential benefits of treatment withdrawal. Until validated biomarkers or clinical tools for individualized risk stratification emerge, the default approach should prioritize sustained disease suppression.