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As the autoantibody to aquaporin-4 (AQP4-Ab) can mediate the pathogenesis of NMOSD, testing for the AQP4-Ab in serum of patients can play a crucial role in diagnosing NMOSD. Nevertheless, the differential diagnosis of NMOSD in clinical practice is often challenging despite the phenotypical and serological characteristics of the disease because: (1) diverse diseases with autoimmune, vascular, infectious, or neoplastic etiologies can mimic these phenotypes of NMOSD; (2) patients with NMOSD may only have limited clinical manifestations, especially in their early disease stages; (3) test results for AQP4-Ab can be affected by several factors such as assay methods, serologic status, disease stages, or types of treatment; (4) some patients with NMOSD do not have AQP4-Ab; and (5) test results for the AQP4-Ab may not be readily available for the acute management of patients. Despite some similarity in their phenotypes, these NMOSD and NMOSD-mimics are distinct from each other in their pathogenesis, prognosis, and most importantly treatment. Understanding the detailed clinical, serological, radiological, and prognostic differences of these diseases will improve the proper management as well as diagnosis of patients. <\/jats:p>","DOI":"10.1177\/1756285617709723","type":"journal-article","created":{"date-parts":[[2017,5,25]],"date-time":"2017-05-25T04:05:07Z","timestamp":1495685107000},"page":"265-289","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":92,"title":["Differential diagnosis of neuromyelitis optica spectrum disorders"],"prefix":"10.1177","volume":"10","author":[{"given":"Sung-Min","family":"Kim","sequence":"first","affiliation":[{"name":"Department of Neurology, Seoul National University Hospital, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seong-Joon","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Seoul National University, College of Medicine, Seoul, 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