title: Enhancing Brain Lesions during Acute Optic Neuritis and/or Longitudinally Extensive Transverse Myelitis May Portend a Higher Relapse Rate in Neuromyelitis Optica Spectrum Disorders.
abstract: OBJECTIVE
  Neuromyelitis optica spectrum disorders are inflammatory demyelinating disorders with optic neuritis and/or longitudinally extensive transverse myelitis episodes. We now know that neuromyelitis optica spectrum disorders are associated with antibodies to aquaporin-4, which are highly concentrated on astrocytic end-feet at the blood-brain barrier. Immune-mediated disruption of the blood-brain barrier may manifest as contrast enhancement on brain MR imaging. We aimed to delineate the extent and frequency of contrast enhancement on brain MR imaging within 1 month of optic neuritis and/or longitudinally extensive transverse myelitis attacks and to correlate contrast enhancement with outcome measures.
  
  
  METHODS
  Brain MRIs of patients with neuromyelitis optica spectrum disorders were evaluated for patterns of contrast enhancement (periependymal, cloudlike, leptomeningeal, and so forth). The Fisher exact test was used to evaluate differences between the proportion of contrast enhancement in patients who were seropositive and seronegative for aquaporin-4 antibodies. The Mann-Whitney test was used to compare the annualized relapse rate and disease duration between patients with and without contrast enhancement and with and without seropositivity.
  
  
  RESULTS
  Brain MRIs of 77 patients were evaluated; 59 patients (10 males, 49 females) were scanned within 1 month of optic neuritis and/or longitudinally extensive transverse myelitis attacks and were included in the analysis. Forty-eight patients were seropositive, 9 were seronegative, and 2 were not tested for aquaporin-4 antibodies. Having brain contrast enhancement of any type during an acute attack was significantly associated with higher annualized relapse rates (P= .03) and marginally associated with shorter disease duration (P= .05). Having periependymal contrast enhancement was significantly associated with higher annualized relapse rates (P= .03).
  
  
  CONCLUSIONS
  Brain MRIs of patients with neuromyelitis optica spectrum disorders with contrast enhancement during an acute relapse of optic neuritis and/or longitudinally extensive transverse myelitis are associated with increased annual relapse rates.
authors.affiliation: From the Division of Neuroradiology (G.O., Y.P., I.I.), Russell H. Morgan Department of Radiology.
authors.forename: G
authors.identifier: http://orcid.org/0000-0003-2684-3957
authors.initials: G
authors.lastname: Orman
authors.affiliation: Department of Radiology (K.Y.W.), Baylor College of Medicine, Houston, Texas.
authors.forename: K Y
authors.identifier: http://orcid.org/0000-0002-2995-2975
authors.initials: KY
authors.lastname: Wang
authors.affiliation: From the Division of Neuroradiology (G.O., Y.P., I.I.), Russell H. Morgan Department of Radiology.
authors.forename: Y
authors.identifier: http://orcid.org/0000-0003-1421-3376
authors.initials: Y
authors.lastname: Pekcevik
authors.affiliation: Biostatistics Center (C.B.T.), Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland.
authors.forename: C B
authors.identifier: http://orcid.org/0000-0003-2792-9250
authors.initials: CB
authors.lastname: Thompson
authors.affiliation: Department of Neurology (M.M., M.L.), The Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: M
authors.identifier: http://orcid.org/0000-0001-8967-6338
authors.initials: M
authors.lastname: Mealy
authors.affiliation: Department of Neurology (M.M., M.L.), The Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: M
authors.identifier: http://orcid.org/0000-0002-7969-8346
authors.initials: M
authors.lastname: Levy
authors.affiliation: From the Division of Neuroradiology (G.O., Y.P., I.I.), Russell H. Morgan Department of Radiology iizbuda1@jhmi.edu.
authors.forename: I
authors.identifier: http://orcid.org/0000-0002-9845-5171
authors.initials: I
authors.lastname: Izbudak
mesh_terms: D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging* / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D059906:Neuroimaging; D009471:Neuromyelitis Optica / Q000000981:diagnostic imaging* / Q000473:pathology*; D012008:Recurrence