Relapsing anti-aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder diagnosed double positive for anti-myelin oligodendrocyte glycoprotein antibody

Neuromyelitis optica spectrum disorder (NMOSD) is associated with anti-aquaporin-4 antibodies (AQP4-Ab), whereas myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is associated with anti-myelin oligodendrocyte glycoprotein antibodies (MOG-Ab). Cases in which both antibodies coexist are rare. We describe a clinical case diagnosed with AQP4-Ab-positive NMOSD 7 years prior, who exhibited new symptoms that were similar to those of MOGAD during her third attack. She was ultimately diagnosed as being double positive for AQP4-Ab and MOG-Ab. Although a full treatment of steroid pulse and plasma exchange was administered, the patient had severe vision loss. Double-antibody-positive cases are very rare in NMOSD patients; however, the investigation of multiple possibilities remains important. When patients experience new symptoms or treatment responses during attacks, a new antibody association should always be considered.