title: Characteristic brain magnetic resonance imaging abnormalities in central nervous system aquaporin-4 autoimmunity.
abstract: BACKGROUND
  Although neuromyelitis optica has been traditionally regarded as a disease without brain involvement, brain abnormalities are not uncommon in patients with neuromyelitis optica-related disorders.
  
  
  METHODS
  We aimed to characterize the brain magnetic resonance imaging (MRI) abnormalities in neuromyelitis optica spectrum disorder patients who are seropositive for anti-aquaporin-4 autoantibody (AQP4 Ab). Of 236 consecutive patients with inflammatory demyelinating central nervous system diseases, we retrospectively analyzed MRI characteristics of 78 patients who were seropositive for AQP4 Ab.
  
  
  RESULTS
  For an average observational period of 6.3 years, 62 patients (79%) had brain lesions on MRI. Twenty-four patients (31%) had brain MRI abnormalities at the onset of disease, and 35 (45%) had symptomatic brain involvement. Characteristic brain MRI abnormalities were classified into five categories: (1) lesions involving corticospinal tracts (e.g. posterior limb of internal capsule and cerebral peduncle (44%); (2) extensive hemispheric lesions likely due to vasogenic edema (29%); (3) periependymal lesions surrounding aqueduct and the third and fourth ventricles (22%); (4) periependymal lesions surrounding lateral ventricles (40%); and (5) medullary lesions, often contiguous with cervical lesions (31%). Fifty-four patients (69%) showed at least one kind of brain abnormality among the five characteristic MRI lesions. Ten patients showed gadolinium-enhancing lesions, which were characterized by multiple patchy enhancing patterns with blurred margins.
  
  
  CONCLUSIONS
  In central nervous system AQP4 autoimmunity, brain MRI abnormalities were more common than is generally appreciated and were characterized by their unique localization and configuration.
authors.affiliation: Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors.forename: Woojun
authors.initials: W
authors.lastname: Kim
authors.forename: Min Su
authors.initials: MS
authors.lastname: Park
authors.forename: Sang Hyun
authors.initials: SH
authors.lastname: Lee
authors.forename: Su-Hyun
authors.initials: SH
authors.lastname: Kim
authors.forename: In Ja
authors.initials: IJ
authors.lastname: Jung
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Takahashi
authors.forename: Tatsuro
authors.initials: T
authors.lastname: Misu
authors.forename: Kazuo
authors.initials: K
authors.lastname: Fujihara
authors.forename: Ho Jin
authors.initials: HJ
authors.lastname: Kim
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D051401:Aquaporin 4 / Q000276:immunology*; D001327:Autoimmune Diseases / Q000276:immunology* / Q000473:pathology*; D001921:Brain / Q000473:pathology*; D003287:Contrast Media; D020278:Demyelinating Autoimmune Diseases, CNS / Q000276:immunology / Q000473:pathology; D004185:Disability Evaluation; D018450:Disease Progression; D005260:Female; D019786:Gadolinium DTPA; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009471:Neuromyelitis Optica / Q000473:pathology; D012008:Recurrence; D012189:Retrospective Studies; D013116:Spinal Cord / Q000473:pathology
chemical_list: D051401:Aquaporin 4; D003287:Contrast Media; D007074:Immunoglobulin G; D019786:Gadolinium DTPA