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The relationship between <jats:styled-content style=\"fixed-case\">NMO<\/jats:styled-content> and multiple sclerosis (<jats:styled-content style=\"fixed-case\">MS<\/jats:styled-content>) has long been a matter of debate. However, the discovery of an <jats:styled-content style=\"fixed-case\">NMO<\/jats:styled-content>\u2010specific autoantibody, <jats:styled-content style=\"fixed-case\">NMO<\/jats:styled-content>\u2010immunoglobulin <jats:styled-content style=\"fixed-case\">G<\/jats:styled-content>\/aquaporin 4 (<jats:styled-content style=\"fixed-case\">AQP4<\/jats:styled-content>) antibody, has dramatically advanced our understanding of the disease, and the clinical, magnetic resonance imaging (<jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>), optical coherence tomography, and laboratory examinations have clarified unique features of <jats:styled-content style=\"fixed-case\">NMO<\/jats:styled-content> that are distinct from <jats:styled-content style=\"fixed-case\">MS<\/jats:styled-content>. The term <jats:styled-content style=\"fixed-case\">NMO<\/jats:styled-content> spectrum disorders (<jats:styled-content style=\"fixed-case\">NMOSD<\/jats:styled-content>) incorporating spatially limited forms was introduced, as patients with recurrent or simultaneous bilateral <jats:styled-content style=\"fixed-case\">ON<\/jats:styled-content> or recurrent longitudinally extensive <jats:styled-content style=\"fixed-case\">TM<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">LETM<\/jats:styled-content>) alone are also often <jats:styled-content style=\"fixed-case\">AQP4<\/jats:styled-content> antibody\u2010seropositive. Moreover, studies of seropositive cases have shown that more than half of them have brain lesions, some of which are unique to <jats:styled-content style=\"fixed-case\">NMO<\/jats:styled-content>, and can be the onset manifestation. Some clinical features of <jats:styled-content style=\"fixed-case\">AQP4<\/jats:styled-content> antibody\u2010seronegative <jats:styled-content style=\"fixed-case\">NMO<\/jats:styled-content> differ from seropositive, but it remains unknown whether they are pathologically distinct. Immunosuppressive treatments are effective for acute attacks and prevention of relapses of <jats:styled-content style=\"fixed-case\">NMOSD<\/jats:styled-content>, and new molecularly targeted drugs are under investigation. Importantly, some disease modifying drugs for <jats:styled-content style=\"fixed-case\">MS<\/jats:styled-content> may exacerbate <jats:styled-content style=\"fixed-case\">NMOSD<\/jats:styled-content>, making early differential diagnosis of the two diseases crucial. We review the evolving clinical spectrum, the updated clinical, <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>, neuro\u2010ophthalmological and laboratory findings, and the current status of treatment in <jats:styled-content style=\"fixed-case\">NMOSD<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/bpa.12087","type":"journal-article","created":{"date-parts":[[2013,10,14]],"date-time":"2013-10-14T06:19:27Z","timestamp":1381731567000},"page":"647-660","source":"Crossref","is-referenced-by-count":43,"title":["Clinical Spectrum and Treatment of Neuromyelitis Optica Spectrum Disorders: Evolution and Current Status"],"prefix":"10.1111","volume":"23","author":[{"given":"Douglas Kazutoshi","family":"Sato","sequence":"first","affiliation":[{"name":"Department of Neurology Tohoku University Graduate School of Medicine  Sendai Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco Aurelio","family":"Lana\u2010Peixoto","sequence":"additional","affiliation":[{"name":"CIEM MS Research Center Federal University of Minas Gerais Medical School  Belo Horizonte Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuo","family":"Fujihara","sequence":"additional","affiliation":[{"name":"Department of Multiple Sclerosis Therapeutics Tohoku University Graduate School of Medicine  Sendai Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jerome","family":"de Seze","sequence":"additional","affiliation":[{"name":"Department of Neurology Strasbourg University and Clinical Investigation Center Strasbourg Hospital  Strasbourg France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,10,14]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1177\/1352458509353651"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22121"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.3109\/s10165-012-0715-9"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3182190f74"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1177\/1352458511417480"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2013.1246"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1177\/1352458511421185"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1177\/1352458511404586"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneurol.2010.203"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneurol.2012.545"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncprheum0818"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e31824c466f"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1177\/1352458513476562"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.21837"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1177\/1352458509350741"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0058925"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004150070172"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.56.4.507"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2012-303121"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.2011.03565.x"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-009-0009-0"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1017385108"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e318207b1f6"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1590\/S0004-27492007000100029"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e31822a2780"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20851"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.65.7.920"},{"key":"e_1_2_7_29_1","first-page":"1033","article-title":"Myelite subaigu\u00eb compliqu\u00e9e de n\u00e9vrite optique","volume":"8","author":"Devic E","year":"1894","journal-title":"Bull Med"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1177\/1352458512457720"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1097\/WNO.0b013e31823a9ebc"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2005.10.040"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e318296ea22"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1759-1961.2012.00030.x"},{"key":"e_1_2_7_35_1","unstructured":"GaultF(1894)De la neuromy\u00e9lite optique aigu\u00eb. 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