title: Neuromyelitis optica spectrum disorders with aquaporin-4 and myelin-oligodendrocyte glycoprotein antibodies: a comparative study.
abstract: OBJECTIVE
  Most patients with neuromyelitis optica (NMO) and many with NMO spectrum disorder have autoantibodies against aquaporin-4 (AQP4-Abs), but recently, myelin-oligodendrocyte glycoprotein antibodies (MOG-Abs) have been found in some patients. Here, we showed that patients with NMO/NMOSD with MOG-Abs demonstrate differences when compared with patients with AQP4-Abs.
  
  
  OBJECTIVE
  To characterize the features of patients with NMO/NMOSD with MOG-Abs and compare them with patients with AQP4-Ab-positive NMO/NMOSD.
  
  
  METHODS
  This observational study was conducted at a single UK specialist center for NMO. Patients with a first demyelinating event between January 1, 2010, and April 1, 2013, seen within the Oxford NMO service and who tested positive for MOG-Abs or AQP4-Abs were included in the study.
  
  
  METHODS
  Cell-based assays using C-terminal-truncated human MOG and full-length M23-AQP4 were used to test patient serum samples for AQP4-Abs and MOG-Abs.
  
  
  METHODS
  Demographic, clinical, and disability data, and magnetic resonance imaging findings.
  
  
  RESULTS
  Twenty AQP4-Ab-positive patients and 9 MOG-Ab-positive patients were identified. Most patients in both groups were white. Ninety percent of AQP4-Ab-positive patients but only 44% MOG-Ab-positive patients were females (P = .02) with a trend toward older age at disease onset in AQP4-Ab-positive patients (44.9 vs 32.3 years; P = .05). MOG-Ab-positive patients more frequently presented with simultaneous/sequential optic neuritis and myelitis (44% vs 0%; P = .005). Onset episode severity did not differ between the 2 groups, but patients with MOG-Abs had better outcomes from the onset episode, with better recovery Expanded Disability Status Scale scores and a lower risk for visual and motor disability. Myelin-oligodendrocyte glycoprotein antibody-positive patients were more likely to have conus involvement on spinal magnetic resonance imaging (75% vs 17%; P = .02) and involvement of deep gray nuclei on brain magnetic resonance imaging (P = .03). Cerebrospinal fluid characteristics were similar in the 2 groups. A higher proportion of AQP4-Ab-positive patients relapsed (40% vs 0%; P = .03) despite similar follow-up durations.
  
  
  CONCLUSIONS
  Despite the fact that patients with MOG-Abs can fulfill the diagnostic criteria for NMO, there are differences when compared with those with AQP4-Abs. These include a higher proportion of males, younger age, and greater likelihood of involvement of the conus and deep gray matter structures on imaging. Additionally, patients with MOG-Abs had more favorable outcomes. Patients with AQP4-Ab-negative NMO/NMOSD should be tested for MOG-Abs.
authors.affiliation: Nuffield Department of Clinical Neurosciences, Oxford University Hospitals National Health Service Trust, University of Oxford, Oxford, England.
authors.forename: Joanna
authors.initials: J
authors.lastname: Kitley
authors.affiliation: Nuffield Department of Clinical Neurosciences, Oxford University Hospitals National Health Service Trust, University of Oxford, Oxford, England.
authors.forename: Patrick
authors.initials: P
authors.lastname: Waters
authors.affiliation: Nuffield Department of Clinical Neurosciences, Oxford University Hospitals National Health Service Trust, University of Oxford, Oxford, England.
authors.forename: Mark
authors.initials: M
authors.lastname: Woodhall
authors.affiliation: Nuffield Department of Clinical Neurosciences, Oxford University Hospitals National Health Service Trust, University of Oxford, Oxford, England.
authors.forename: M Isabel
authors.initials: MI
authors.lastname: Leite
authors.affiliation: Nuffield Department of Clinical Neurosciences, Oxford University Hospitals National Health Service Trust, University of Oxford, Oxford, England.
authors.forename: Andrew
authors.initials: A
authors.lastname: Murchison
authors.affiliation: Nuffield Department of Clinical Neurosciences, Oxford University Hospitals National Health Service Trust, University of Oxford, Oxford, England.
authors.forename: Jithin
authors.initials: J
authors.lastname: George
authors.affiliation: Department of Neuroradiology, Oxford University Hospitals National Health Service Trust, Oxford, England.
authors.forename: Wilhelm
authors.initials: W
authors.lastname: Küker
authors.affiliation: Children's Hospital, Oxford University Hospitals National Health Service Trust, Oxford, England.
authors.forename: Saleel
authors.initials: S
authors.lastname: Chandratre
authors.affiliation: Nuffield Department of Clinical Neurosciences, Oxford University Hospitals National Health Service Trust, University of Oxford, Oxford, England.
authors.forename: Angela
authors.initials: A
authors.lastname: Vincent
authors.affiliation: Nuffield Department of Clinical Neurosciences, Oxford University Hospitals National Health Service Trust, University of Oxford, Oxford, England.
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Palace
mesh_terms: D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000429:Alcohol Oxidoreductases; D051401:Aquaporin 4 / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D004268:DNA-Binding Proteins; D004185:Disability Evaluation; D005260:Female; D005500:Follow-Up Studies; D006629:Hirudins; D006801:Humans; D008297:Male; D008875:Middle Aged; D063308:Myelin-Oligodendrocyte Glycoprotein / Q000276:immunology*; D009471:Neuromyelitis Optica / Q000097:blood / Q000134:cerebrospinal fluid / Q000276:immunology* / Q000473:pathology; D011994:Recombinant Proteins; D012720:Severity of Illness Index; D012737:Sex Factors; D014568:Urokinase-Type Plasminogen Activator
chemical_list: D051401:Aquaporin 4; D001323:Autoantibodies; D004268:DNA-Binding Proteins; D006629:Hirudins; C103494:M23 urokinase; D063308:Myelin-Oligodendrocyte Glycoprotein; D011994:Recombinant Proteins; D000429:Alcohol Oxidoreductases; C096794:C-terminal binding protein; D014568:Urokinase-Type Plasminogen Activator