{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T19:21:37Z","timestamp":1792178497539,"version":"4.4.0"},"reference-count":291,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2014,4,9]],"date-time":"2014-04-09T00:00:00Z","timestamp":1397001600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Merck Serono"},{"DOI":"10.13039\/100008659","name":"European Committee for Treatment and Research in Multiple Sclerosis","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100008659","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2014,4,9]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>The term \u2018neuromyelitis optica\u2019 (\u2018Devic's syndrome\u2019, NMO) refers to a syndrome characterized by optic neuritis and myelitis. In recent years, the condition has raised enormous interest among scientists and clinical neurologists, fuelled by the detection of a specific serum immunoglobulin (Ig)G reactivity (NMO-IgG) in up to 80% of patients with NMO. These autoantibodies were later shown to target aquaporin-4 (AQP4), the most abundant water channel in the central nervous system (CNS). Here we give an up-to-date overview of the clinical and paraclinical features, immunopathogenesis and treatment of NMO. We discuss the widening clinical spectrum of AQP4-related autoimmunity, the role of magnetic resonance imaging (MRI) and new diagnostic means such as optical coherence tomography in the diagnosis of NMO, the role of NMO-IgG, T cells and granulocytes in the pathophysiology of NMO, and outline prospects for new and emerging therapies for this rare, but often devastating condition.<\/jats:p>","DOI":"10.1111\/cei.12271","type":"journal-article","created":{"date-parts":[[2014,1,13]],"date-time":"2014-01-13T08:11:11Z","timestamp":1389600671000},"page":"149-164","source":"Crossref","is-referenced-by-count":309,"title":["Neuromyelitis optica: clinical features, immunopathogenesis and treatment"],"prefix":"10.1093","volume":"176","author":[{"given":"S","family":"Jarius","sequence":"first","affiliation":[{"name":"Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B","family":"Wildemann","sequence":"additional","affiliation":[{"name":"Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F","family":"Paul","sequence":"additional","affiliation":[{"name":"NeuroCure Clinical Research Center and Clinical and Experimental Multiple Sclerosis Research Center, Department of Neurology, Charit\u00e9 University Medicine, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2014,4,9]]},"reference":[{"key":"2021122010031329300_cei12271-bib-0001","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1186\/1742-2094-9-14","article-title":"Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: a multicentre study of 175 patients","volume":"9","author":"Jarius","year":"2012","journal-title":"J Neuroinflammation"},{"key":"2021122010031329300_cei12271-bib-0002","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1007\/s00115-012-3602-x","article-title":"[Neuromyelitis optica]","volume":"84","author":"Wildemann","year":"2013","journal-title":"Nervenarzt"},{"key":"2021122010031329300_cei12271-bib-0003","doi-asserted-by":"crossref","first-page":"1365","DOI":"10.1007\/s00115-007-2262-8","article-title":"[Neuromyelitis optica]","volume":"78","author":"Jarius","year":"2007","journal-title":"Nervenarzt"},{"key":"2021122010031329300_cei12271-bib-0004","doi-asserted-by":"crossref","first-page":"768","DOI":"10.1007\/s00115-010-3192-4","article-title":"[Diagnosis and treatment of neuromyelitis optica. Consensus recommendations of the Neuromyelitis Optica Study Group]","volume":"82","author":"Trebst","year":"2011","journal-title":"Nervenarzt"},{"key":"2021122010031329300_cei12271-bib-0005","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1084\/jem.20050304","article-title":"IgG marker of optic\u2013spinal multiple sclerosis binds to the aquaporin-4 water channel","volume":"202","author":"Lennon","year":"2005","journal-title":"J Exp Med"},{"key":"2021122010031329300_cei12271-bib-0006","doi-asserted-by":"crossref","first-page":"2106","DOI":"10.1016\/S0140-6736(04)17551-X","article-title":"A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis","volume":"364","author":"Lennon","year":"2004","journal-title":"Lancet"},{"key":"2021122010031329300_cei12271-bib-0007","doi-asserted-by":"crossref","first-page":"1076","DOI":"10.1212\/01.wnl.0000256822.01222.bd","article-title":"NMO-IgG in the diagnosis of neuromyelitis optica","volume":"68","author":"Jarius","year":"2007","journal-title":"Neurology"},{"key":"2021122010031329300_cei12271-bib-0008","doi-asserted-by":"crossref","first-page":"e133","DOI":"10.1371\/journal.pmed.0040133","article-title":"Antibody to aquaporin 4 in the diagnosis of neuromyelitis optica","volume":"4","author":"Paul","year":"2007","journal-title":"PLOS Med"},{"key":"2021122010031329300_cei12271-bib-0009","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.jns.2010.01.002","article-title":"Standardized method for the detection of antibodies to aquaporin-4 based on a highly sensitive immunofluorescence assay employing recombinant target antigen","volume":"291","author":"Jarius","year":"2010","journal-title":"J Neurol Sci"},{"key":"2021122010031329300_cei12271-bib-0010","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1016\/j.jns.2012.06.002","article-title":"Testing for antibodies to human aquaporin-4 by ELISA: sensitivity, specificity, and direct comparison with immunohistochemistry","volume":"320","author":"Jarius","year":"2012","journal-title":"J Neurol Sci"},{"key":"2021122010031329300_cei12271-bib-0011","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1001\/archneur.65.7.913","article-title":"Aquaporin-4 antibodies in neuromyelitis optica and longitudinally extensive transverse myelitis","volume":"65","author":"Waters","year":"2008","journal-title":"Arch Neurol"},{"key":"2021122010031329300_cei12271-bib-0012","doi-asserted-by":"crossref","first-page":"1450","DOI":"10.1093\/brain\/awf151","article-title":"A role for humoral mechanisms in the pathogenesis of Devic's neuromyelitis optica","volume":"125","author":"Lucchinetti","year":"2002","journal-title":"Brain"},{"key":"2021122010031329300_cei12271-bib-0013","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1016\/S1474-4422(12)70301-0","article-title":"The expanding range of autoimmune disorders of the nervous system","volume":"12","author":"Wildemann","year":"2013","journal-title":"Lancet Neurol"},{"key":"2021122010031329300_cei12271-bib-0014","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1007\/s00115-012-3601-y","article-title":"[Immune-mediated encephalomyelitis]","volume":"84","author":"Wildemann","year":"2013","journal-title":"Nervenarzt"},{"key":"2021122010031329300_cei12271-bib-0015","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1016\/S1474-4422(07)70216-8","article-title":"The spectrum of neuromyelitis optica","volume":"6","author":"Wingerchuk","year":"2007","journal-title":"Lancet Neurol"},{"key":"2021122010031329300_cei12271-bib-0016","volume-title":"Changing concepts in CNS demyelinating disorders: autoimmune water channelopathies","author":"Lennon","year":"2006"},{"key":"2021122010031329300_cei12271-bib-0017","doi-asserted-by":"crossref","first-page":"834","DOI":"10.1177\/1352458509104595","article-title":"Aquaporin-4 autoimmune syndrome and anti-aquaporin-4 antibody-negative opticospinal multiple sclerosis in Japanese","volume":"15","author":"Matsushita","year":"2009","journal-title":"Mult Scler"},{"key":"2021122010031329300_cei12271-bib-0018","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1186\/1742-2094-10-8","article-title":"The history of neuromyelitis optica","volume":"10","author":"Jarius","year":"2013","journal-title":"J Neuroinflammation"},{"key":"2021122010031329300_cei12271-bib-0019","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1016\/j.jns.2011.09.025","article-title":"\u2018Noteomielite\u2019 accompanied by acute amaurosis (1844). 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