{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T23:51:16Z","timestamp":1790639476038,"version":"4.1.0"},"reference-count":37,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2023,7,19]],"date-time":"2023-07-19T00:00:00Z","timestamp":1689724800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2023,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Differentiating neuromyelitis optica spectrum disorder (NMOSD) from its mimics is crucial to avoid misdiagnosis, especially in the absence of aquaporin\u20104\u2010IgG. While multiple sclerosis (MS) and myelin oligodendrocyte glycoprotein\u2010IgG associated disease (MOGAD) represent major and well\u2010defined differential diagnoses, non\u2010demyelinating NMOSD mimics remain poorly characterized.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a systematic review on PubMed\/MEDLINE to identify reports of patients with non\u2010demyelinating disorders that mimicked or were misdiagnosed as NMOSD. Three novel cases seen at the authors' institutions were also included. The characteristics of NMOSD mimics were analyzed and red flags associated with misdiagnosis identified.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      A total of 68 patients were included; 35 (52%) were female. Median age at symptoms onset was 44\u2009(range, 1\u201378) years. Fifty\u2010six (82%) patients did not fulfil the 2015 NMOSD diagnostic criteria. The clinical syndromes misinterpreted for NMOSD were myelopathy (41%), myelopathy\u2009+\u2009optic neuropathy (41%), optic neuropathy (6%), or other (12%). Alternative etiologies included genetic\/metabolic disorders, neoplasms, infections, vascular disorders, spondylosis, and other immune\u2010mediated disorders. Common red flags associated with misdiagnosis were lack of cerebrospinal fluid (CSF) pleocytosis (57%), lack of response to immunotherapy (55%), progressive disease course (54%), and lack of magnetic resonance imaging gadolinium enhancement (31%). Aquaporin\u20104\u2010IgG positivity was detected in five patients by enzyme\u2010linked immunosorbent assay (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20092), cell\u2010based assay (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20092: serum, 1; CSF, 1), and non\u2010specified assay (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The spectrum of NMOSD mimics is broad. Misdiagnosis frequently results from incorrect application of diagnostic criteria, in patients with multiple identifiable red flags. False aquaporin\u20104\u2010IgG positivity, generally from nonspecific testing assays, may rarely contribute to misdiagnosis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/ene.15983","type":"journal-article","created":{"date-parts":[[2023,7,11]],"date-time":"2023-07-11T20:34:56Z","timestamp":1689107696000},"page":"3367-3376","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Non\u2010demyelinating disorders mimicking and misdiagnosed as\n                    <scp>NMOSD:<\/scp>\n                    a literature review"],"prefix":"10.1111","volume":"30","author":[{"given":"Pietro","family":"Zara","sequence":"first","affiliation":[{"name":"Neurology Unit, Department of Medical, Surgical and Experimental Sciences University of Sassari Sassari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3249-4792","authenticated-orcid":false,"given":"Alessandro","family":"Dinoto","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Carta","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Floris","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Medical, Surgical and Experimental Sciences University of Sassari Sassari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davide","family":"Turilli","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Medical, Surgical and Experimental Sciences University of Sassari Sassari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adrian","family":"Budhram","sequence":"additional","affiliation":[{"name":"Department of Clinical Neurological Sciences Western University London Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3855-5135","authenticated-orcid":false,"given":"Sergio","family":"Ferrari","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefania","family":"Milia","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Medical, Surgical and Experimental Sciences University of Sassari Sassari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Solla","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Medical, Surgical and Experimental Sciences University of Sassari Sassari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7806-3103","authenticated-orcid":false,"given":"Sara","family":"Mariotto","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6661-2910","authenticated-orcid":false,"given":"Eoin P.","family":"Flanagan","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic Rochester Minnesota USA"},{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. 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