Double-seronegative neuromyelitis optica: it is possible to interrupt treatment after 10 years of stability

Double-seronegative neuromyelitis optica spectrum disorder (DS-NMOSD) encompasses a heterogeneous spectrum, including monophasic and relapsing phenotypes. While 1/3 patients may follow a monophasic course, lifelong immunotherapy remains common practice due to the fear of relapse. However, this strategy may unnecessarily expose stable patients to long-term adverse effects and economic burden. The condition lacks the relapse-associated mechanisms observed in aquaporin 4 (AQP4)-positive disease, questioning the generalizability of prior treatment-withdrawal studies. Although predictive markers for relapse are still lacking, the median time to relapse is of approximately 3.4 (range: 0-7) years; hence, sustained remission beyond 10 years may indicate a subgroup of patients with low relapse risk. Until prospective data are available, individualized, cautious treatment interruption should be considered, guided by shared decision-making.