title: Cerebral Cortex Involvement in Neuromyelitis Optica Spectrum Disorder.
abstract: OBJECTIVE
  Brain lesions involving the cerebral cortex are rarely described in patients with neuromyelitis optica spectrum disorder (NMOSD), in contrast to multiple sclerosis. We investigated cerebral cortex involvement using conventional brain magnetic resonance imaging (MRI) in anti-aquaporin-4 (AQP4)-antibody-positive NMOSD patients.
  
  
  METHODS
  The study enrolled 215 NMOSD patients who were seropositive for the anti-AQP4 antibody from 5 referral hospitals, and retrospectively analyzed their demographic, clinical, and MRI findings. Abnormal cerebral cortex lesions on brain MRI were identified by a neuroradiologist and two neurologists using consensus.
  
  
  RESULTS
  Most of the 215 enrolled patients (87%) were female. The median age at onset was 22.5 years (range: 15-36 years) and the mean follow-up duration was 123 months. Brain lesions were found in 143 of 194 patients (74%) in whom MRI was performed during follow-up. Brain lesions involving the cerebral cortex were identified in 6 of these 194 patients (3.1%). Five of the patients were female, and the six patients together had a median age of 29 years (range: 15-36 years) at the time of lesion presentation. Three of them showed leptomeningeal enhancement in the lesions. At presentation of the cortex-involving lesions, five of these patients were not being treated at the time of presentation, while the sixth was being treated with interferon-beta.
  
  
  CONCLUSIONS
  Although rare, cortical involvement occurs in NMOSD and is commonly combined with leptomeningeal enhancement. We speculate that this occurs only in patients who are not treated appropriately with immunosuppressant drugs.
authors.affiliation: Department of Neurology, The Catholic University of Korea College of Medicine, Seoul, Korea. wjkim@catholic.ac.kr.
authors.forename: Woojun
authors.initials: W
authors.lastname: Kim
authors.affiliation: Department of Neurology, The Catholic University of Korea College of Medicine, Seoul, Korea.
authors.forename: Jee Eun
authors.initials: JE
authors.lastname: Lee
authors.affiliation: Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors.forename: Su Hyun
authors.initials: SH
authors.lastname: Kim
authors.affiliation: Department of Neurology, Kosin University College of Medicine, Busan, Korea.
authors.forename: So Young
authors.initials: SY
authors.lastname: Huh
authors.affiliation: Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors.forename: Jae Won
authors.initials: JW
authors.lastname: Hyun
authors.affiliation: Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors.forename: In Hye
authors.initials: IH
authors.lastname: Jeong
authors.affiliation: Department of Neurology, Yeungnam University College of Medicine, Daegu, Korea.
authors.forename: Min Su
authors.initials: MS
authors.lastname: Park
authors.affiliation: Department of Neurology, Inje University Ilsan Paik Hospital, Goyang, Korea.
authors.forename: Joong Yang
authors.initials: JY
authors.lastname: Cho
authors.affiliation: Department of Radiolgoy, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors.forename: Sang Hyun
authors.initials: SH
authors.lastname: Lee
authors.affiliation: Department of Neurology, The Catholic University of Korea College of Medicine, Seoul, Korea.
authors.forename: Kwang Soo
authors.initials: KS
authors.lastname: Lee
authors.affiliation: Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors.forename: Ho Jin
authors.initials: HJ
authors.lastname: Kim
keywords: cerebral cortex; magnetic resonance imaging; neuromyelitis optica; neuromyelitis optica spectrum disorder
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