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The heterogeneity of clinical presentations and wide range of differential diagnoses often lead to missed diagnoses of NMOSD. Misapplication of the 2015 NMOSD criteria and misinterpretation of clinical and neuroradiological findings are relevant factors associated with misdiagnosis in clinical practice. Despite the presence of a specific biomarker for NMOSD (AQP4-IgG), misdiagnosis rates have been reported as high as 35%. Studies indicate that misdiagnosed patients often undergo unnecessary prolonged immunotherapy, leading to health risks and increased morbidity. Accurate definitive diagnosis is crucial as long-term outcomes and treatment approaches differ based on the correct diagnosis, and inappropriate immunotherapy can lead to disability in NMOSD patients. This review outlines factors linked to NMOSD misdiagnosis and briefly discusses strategies to reduce misdiagnosis.<\/jats:p>","DOI":"10.1177\/13524585241292797","type":"journal-article","created":{"date-parts":[[2024,11,6]],"date-time":"2024-11-06T02:06:23Z","timestamp":1730858783000},"page":"8-22","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":15,"title":["How to avoid missing a diagnosis of neuromyelitis optica spectrum disorder"],"prefix":"10.1177","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7435-5726","authenticated-orcid":false,"given":"Edgar","family":"Carnero Contentti","sequence":"first","affiliation":[{"name":"Neuroimmunology Unit, Department of Neurosciences, Hospital Aleman, Buenos Aires, Argentina"},{"name":"CenRos, Neuroimmunology Clinic, INECO Neurociencias Oro\u00f1o, Rosario, 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