title: Extensive hemispheric lesions with radiological evidence of blood-brain barrier integrity in a patient with neuromyelitis optica.
abstract: Anti-aquaporin-4 antibody (NMO-IgG) is used as a diagnostic marker for neuromyelitis optica (NMO). Although the mechanism of spinal cord lesions in NMO has been investigated, that of extensive hemispheric lesions with brain edema remains unclear. Here we report a 36-year-old woman with NMO positive for NMO-IgG, who developed an acute disseminating encephalomyelitis (ADEM)-like episode after Mycoplasma pneumoniae infection. Brain MRI T2-weighted images demonstrated asymmetric tumefactive hyperintense lesions in the subcortical white matter. Importantly, no lesions on T1-weighted images were enhanced after intravenous gadolinium administration on serial brain MRIs, suggesting preserved integrity of the blood-brain barrier (BBB). Likewise, the corresponding apparent diffusion coefficient maps demonstrated persistent hyperintensity changes, which represented vasogenic edema associated with glial damage and consequent neuronal loss. The findings suggest possible involvement of deficient water elimination associated with seropositivity to NMO-IgG in the induction of vasogenic edema even in the presence of intact and functional BBB.
authors.affiliation: Department of Neurology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan. ssaiki@juntendo.ac.jp
authors.forename: Shinji
authors.initials: S
authors.lastname: Saiki
authors.forename: Yuji
authors.initials: Y
authors.lastname: Ueno
authors.forename: Toshio
authors.initials: T
authors.lastname: Moritani
authors.forename: Takeshi
authors.initials: T
authors.lastname: Sato
authors.forename: Takeshi
authors.initials: T
authors.lastname: Sekine
authors.forename: Sumihiro
authors.initials: S
authors.lastname: Kawajiri
authors.forename: Satoshi
authors.initials: S
authors.lastname: Adachi
authors.forename: Kazumasa
authors.initials: K
authors.lastname: Yokoyama
authors.forename: Yuji
authors.initials: Y
authors.lastname: Tomizawa
authors.forename: Yumiko
authors.initials: Y
authors.lastname: Motoi
authors.forename: Nobutaka
authors.initials: N
authors.lastname: Hattori
mesh_terms: D000328:Adult; D051401:Aquaporin 4 / Q000276:immunology*; D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology*; D001812:Blood-Brain Barrier; D001929:Brain Edema / Q000209:etiology / Q000503:physiopathology; D003287:Contrast Media / Q000493:pharmacokinetics; D003951:Diagnostic Errors; D038524:Diffusion Magnetic Resonance Imaging; D004673:Encephalomyelitis, Acute Disseminated / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D005682:Gadolinium / Q000493:pharmacokinetics; D006801:Humans; D000068576:Interferon beta-1b; D016899:Interferon-beta / Q000009:adverse effects / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D009103:Multiple Sclerosis / Q000175:diagnosis / Q000188:drug therapy; D009177:Mycoplasma pneumoniae; D009188:Myelitis, Transverse / Q000209:etiology; D009471:Neuromyelitis Optica / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000276:immunology / Q000473:pathology; D011019:Pneumonia, Mycoplasma / Q000150:complications*; D011859:Radiography
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies; D001324:Autoantigens; D003287:Contrast Media; D000068576:Interferon beta-1b; D016899:Interferon-beta; D005682:Gadolinium