Neuromyelitis Optica spectrum disorder, formerly known as Devic’s disease, is characterized by an autoimmune-mediated pathology of the central nervous system. This pathology generates an inflammatory process directed against the channels of the protein Aquaporin 4, located mainly in the membrane of astrocytes. Aquaporin-4 is a protein related to water transport, which plays a crucial role in brain inflammation through mechanisms that involve the release of cytokines and astrocytic inflammation. These pathological changes cause demyelination and secondary axonal damage, with diverse clinical manifestations in the brain, spinal cord, and optic nerve sheaths. The detection of antibodies against the protein Aquaporin 4, the analysis of oligoclonal bands in the cerebrospinal fluid, and radiological findings by magnetic resonance imaging of the brain and spinal cord must be carefully evaluated to reach an accurate diagnosis in challenging clinical cases. This report reviews the differential etiologies of this disease and the challenge of comprehensive analysis of the corresponding diagnostic tests.



