Neuromyelitis optica spectrum disorder with AQP4-IgG presenting as area postrema syndrome and progressing to myelitis: A rare case report

KEY CLINICAL MESSAGE: Neuromyelitis optica spectrum disorders can less commonly present with area postrema syndrome progressing to myelitis. Management involves intravenous glucocorticoids, plasma exchange, and preventive immunotherapy. ABSTRACT: Neuromyelitis optica spectrum disorders can less commonly present with area postrema syndrome progressing to myelitis. The majority of patients have positive AQP4-Ab. Diagnosis is based on clinical and imaging findings. These patients can be treated with intravenous glucocorticoids, plasma exchange, and preventive immunotherapy.