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Clinical syndromes are predominated by the relapsing form of NMO but also include encephalopathic variants that can mimic acute disseminated encephalomyelitis. Maintenance immunotherapy is used to prevent relapses in NMO-IgG\u2013seropositive patients. In contrast, NMO-IgG\u2013seronegative children with NMO more commonly have a monophasic course (simultaneous occurrence of optic neuritis and transverse myelitis) and do not require remission-maintaining immunotherapy, but close surveillance is advised. Current clinical, pathological, and pathogenetic knowledge is reviewed with a focus on clinical presentation, neuroimaging findings, serological investigations, and treatment of children with disorders within the spectrum of central nervous system aquaporin-4 autoimmunity.<\/jats:p>","DOI":"10.1177\/0883073812451495","type":"journal-article","created":{"date-parts":[[2012,8,2]],"date-time":"2012-08-02T01:23:27Z","timestamp":1343870607000},"page":"1437-1447","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":33,"title":["The Spectrum of Neuromyelitis Optica (NMO) in Childhood"],"prefix":"10.1177","volume":"27","author":[{"given":"J.M.","family":"Tillema","sequence":"first","affiliation":[{"name":"Department of Neurology, Mayo Clinic, Rochester, MN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"McKeon","sequence":"additional","affiliation":[{"name":"Department of Neurology, Mayo 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