BackgroundNeuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune astrocytopathy that affects several regions of the central nervous system (CNS) with a predilection for the optic nerves and spinal cord. Epidemiological studies of NMOSD are uncommon in the Middle East and up-to-date, there are no such data from Saudi Arabia. In this study, we aim to study the clinical pattern of NMOSD patients in Saudi Arabia. MethodsA retrospective multi-center observational study was conducted at King Abdulaziz Medical City in Jeddah and Riyadh, Saudi Arabia. The inclusion criteria consisted of all the patients with either neuromyelitis optica according to the 2006 criteria or NMOSD according to the 2015 criteria. The study period was 20 years. ResultsA total of 23 patients were included in the study. Four were males (17.4%) and 19 were females (82.6%). The attack type was optic neuritis in 3 patients (13.0%), transverse myelitis in 15 patients (65.2%), and both in 5 patients (21.7%). All patients (100%) received pulse steroid therapy (intravenous methylprednisolone 1 g for 5 days) at the onset of the disease. Fifteen patients had plasma exchange therapy (65.2%). All patients received maintenance immunosuppressive treatment except 1 (4.3%). ConclusionNMOSD is a rare, broad-spectrum, polyphasic, rare disorder primarily affecting the optic pathway and the spinal cord either in isolation or simultaneously. Unfortunately, there are no adequate studies that assess NMOSD cohorts in Saudi Arabia despite the increased number of diagnosed cases. In addition, there is no registry for this disorder with only a few specialized centers dealing with its management. It is time to establish specialized demyelinating disease centers and build expertise in both common and rare diseases in this category.