title: Seronegative Neuromyelitis Optica Spectrum--the challenges on disease definition and pathogenesis.
abstract: Neuromyelitis optica spectrum disorders (NMOSD) are characterized by severe optic neuritis and/or longitudinally extensive transverse myelitis, and some brain lesions are also unique to NMOSD. Serum autoantibodies against aquaporin-4 (AQP4) are detected in most cases of NMOSD. However, some patients with NMOSD remain seronegative despite repetitive testing during attacks with highly sensitive cell-based assays. The differential diagnosis of NMOSD is not restricted to multiple sclerosis and it includes many diseases that can produce longitudinally extensive myelitis and/or optic neuritis. We review the clinical features, imaging, and laboratory findings that can be helpful on the diagnostic work-up, discuss the differences between AQP4 antibody positive and negative patients with NMOSD, including features of NMOSD with antibodies against myelin oligodendrocyte glycoprotein.
authors: Department of Neurology, School of Medicine, Tohoku University, Sendai, Japan.
authors: Douglas Kazutoshi
authors: DK
authors: Sato
authors: Departamento de Neurologia, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
authors: Dagoberto
authors: D
authors: Callegaro
authors: Centro de Investigação em Esclerose Múltipla, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.
authors: Marco Aurélio
authors: MA
authors: Lana-Peixoto
authors: Department of Neurology, School of Medicine, Tohoku University, Sendai, Japan.
authors: Ichiro
authors: I
authors: Nakashima
authors: Department of Multiple Sclerosis Therapeutics, Graduate School of Medicine, Tohoku University, Sendai, Japan.
authors: Kazuo
authors: K
authors: Fujihara
mesh_terms: D000368:Aged; D051401:Aquaporin 4 / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology; D003937:Diagnosis, Differential; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D063308:Myelin-Oligodendrocyte Glycoprotein / Q000276:immunology; D009471:Neuromyelitis Optica / Q000175:diagnosis* / Q000276:immunology*; D013119:Spinal Cord Injuries / Q000175:diagnosis / Q000276:immunology
chemical_list: D051401:Aquaporin 4; D001323:Autoantibodies; D063308:Myelin-Oligodendrocyte Glycoprotein