Treatment De-escalation in AQP4-Ab Neuromyelitis Optica Spectrum Disorder

Aquaporin-4 antibody (AQP4-Ab) neuromyelitis optica spectrum disorder (NMOSD) is a well-recognized autoimmune disease of the CNS. The presence of AQP4-Ab in patients presenting with the core clinical characteristics required for a diagnosis of NMOSD with AQP4-Ab1 is associated with a very high risk of relapse and consequently leads to blindness and paralysis if the syndrome remains untreated.2 Natural history studies have provided the evidence that there is an attack-related stepwise accumulation of disability in patients with NMOSD with AQP4-Ab; therefore, attack prevention strategies are used as maintenance treatment after the first event. The current advice is for lifelong treatment.3