title: A case of pathology-proven neuromyelitis optica spectrum disorder with Sjögren syndrome manifesting aphasia and apraxia due to a localized cerebral white matter lesion.
abstract: A woman with Sjögren syndrome manifesting as aphasia with a left deep cerebral white matter lesion tested positive for anti-aquaporin 4 (AQP4) antibody. Open biopsy of the lesion revealed active demyelination with edematous changes and the preservation of most axons, indicating a non-necrotic demyelinating lesion. Immunostaining for AQP4 was diffusely lost, whereas the loss of glial fibrillary acidic protein immunostaining was limited but with highly degenerated astrocytic foot processes in perivascular areas. These results suggested neuromyelitis optica spectrum disorder (NMOSD) pathology rather than Sjögren-related vasculitis. Only cerebral cortical symptoms with a cerebral white matter lesion could be observed in NMOSDs.
authors.affiliation: Division of Neurology, Asahikawa Medical University, Japan sawadajun@gmail.com.
authors.forename: Jun
authors.initials: J
authors.lastname: Sawada
authors.affiliation: Asahikawa Medical University, Japan.
authors.forename: Ryosuke
authors.initials: R
authors.lastname: Orimoto
authors.affiliation: Tohoku University Graduate School of Medicine, Japan.
authors.forename: Tatsuro
authors.initials: T
authors.lastname: Misu
authors.affiliation: Division of Neurology, Asahikawa Medical University, Japan.
authors.forename: Takayuki
authors.initials: T
authors.lastname: Katayama
authors.affiliation: Tokyo Medical University, Japan.
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Aizawa
authors.affiliation: Division of Neurology, Asahikawa Medical University, Japan.
authors.forename: Asuka
authors.initials: A
authors.lastname: Asanome
authors.affiliation: Division of Neurology, Asahikawa Medical University, Japan.
authors.forename: Kae
authors.initials: K
authors.lastname: Takahashi
authors.affiliation: Division of Neurology, Asahikawa Medical University, Japan.
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Saito
authors.affiliation: Asahikawa Medical University, Japan.
authors.forename: Ryogo
authors.initials: R
authors.lastname: Anei
authors.affiliation: Asahikawa Medical University, Japan.
authors.forename: Kyousuke
authors.initials: K
authors.lastname: Kamada
authors.affiliation: Asahikawa Medical University, Japan.
authors.forename: Naoyuki
authors.initials: N
authors.lastname: Miyokawa
authors.affiliation: Tohoku University Graduate School of Medicine, Japan.
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Takahashi
authors.affiliation: Tohoku University Graduate School of Medicine, Japan.
authors.forename: Kazuo
authors.initials: K
authors.lastname: Fujihara
authors.affiliation: Division of Neurology, Asahikawa Medical University, Japan.
authors.forename: Naoyuki
authors.initials: N
authors.lastname: Hasebe
mesh_terms: D001037:Aphasia / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology; D001072:Apraxias / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology; D051401:Aquaporin 4 / Q000276:immunology; D001323:Autoantibodies / Q000032:analysis; D001706:Biopsy; D002540:Cerebral Cortex / Q000187:drug effects / Q000276:immunology / Q000473:pathology*; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007150:Immunohistochemistry; D056784:Leukoencephalopathies / Q000150:complications / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009471:Neuromyelitis Optica / Q000150:complications / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D016896:Treatment Outcome; D066127:White Matter / Q000187:drug effects / Q000276:immunology / Q000473:pathology*
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies; D005938:Glucocorticoids
keywords: Neuromyelitis optica spectrum disorder (NMOSD); Sjögren syndrome (SS); anti-aquaporin (AQP4) antibody; magnetic resonance imaging (MRI); pathology; white matter lesion