Neuromyelitis optica spectrum disorder is an autoimmune disease of the CNS that primarily affects the optic nerves and spinal cord. Most patients have serum antibodies targeting the aquaporin-4 water channel expressed on the end-feet of astrocytes. Although the prevalence of neuromyelitis optica spectrum disorder is limited to around 1–2 people per 100 000, severe immune-mediated attacks can quickly lead to blindness and paralysis if undiagnosed and untreated. However, diagnosis is straightforward when the highly specific serum aquaporin-4 antibodies are detected with cell-based assays.
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