Extensive hemispheric lesions with radiological evidence of blood-brain barrier integrity in a patient with neuromyelitis optica.
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.
Saiki
Shinji
S
Department of Neurology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan. ssaiki@juntendo.ac.jp
Ueno
Yuji
Y
Moritani
Toshio
T
Sato
Takeshi
T
Sekine
Takeshi
T
Kawajiri
Sumihiro
S
Adachi
Satoshi
S
Yokoyama
Kazumasa
K
Tomizawa
Yuji
Y
Motoi
Yumiko
Y
Hattori
Nobutaka
N
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
C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies; D001324:Autoantigens; D003287:Contrast Media; D000068576:Interferon beta-1b; D016899:Interferon-beta; D005682:Gadolinium