title: Reversible cortical lesions in primary Sjögren's syndrome presenting with meningoencephalitis as an initial manifestation.
abstract: We report a 50-year-old woman with primary Sjögren's syndrome (SjS) who initially showed forgetfulness, and later developed disturbance of consciousness. In addition to aseptic meningoencephalitis revealed by cerebrospinal fluid examination and magnetic resonance imaging (MRI), the presence of serum anti-SS-A and anti-SS-B antibodies and inflammatory findings in lip biopsy indicated primary SjS. Fluid attenuated inversion recovery (FLAIR) of MRI revealed well defined small, high signal intensity areas in the cortex involving the subcortical white matter. Corticosteroid therapy resulted in rapid and nearly complete resolution of the cortical lesions with marked improvement of the clinical manifestations. Memory disturbance is a rare initial manifestation in meningoencephalitis associated with SjS. Our patient with SjS showed inflammatory cortical lesions on MRI, which were reversed by corticosteroid therapy.
authors.affiliation: Department of Neurology and Neurobiology of Aging, Graduate School of Medical Science, Kanazawa University, 13-1 Takara-machi, Kanazawa, Ishikawa 920-8641, Japan.
authors.forename: Mie
authors.initials: M
authors.lastname: Hirohata
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Yasukawa
authors.forename: Chiho
authors.initials: C
authors.lastname: Ishida
authors.forename: Kiyonobu
authors.initials: K
authors.lastname: Komai
authors.forename: Masahito
authors.initials: M
authors.lastname: Yamada
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002540:Cerebral Cortex / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008569:Memory Disorders / Q000209:etiology; D008590:Meningoencephalitis / Q000134:cerebrospinal fluid / Q000188:drug therapy / Q000473:pathology*; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D012859:Sjogren's Syndrome / Q000134:cerebrospinal fluid / Q000188:drug therapy / Q000473:pathology*
chemical_list: D000893:Anti-Inflammatory Agents; D008775:Methylprednisolone