title: Reversible stenosis of large cerebral arteries in a patient with combined Sjögren's syndrome and neuromyelitis optica spectrum disorder.
abstract: We report a 49-year-old woman with neuromyelitis optica (NMO) spectrum disorder coexisting with Sjögren's syndrome (SS). She presented with acute brainstem symptoms and transverse myelitis. Brain MRI showed focal high signal intensity lesions in the hypothalamus and the pontine tegmentum on T2-weighted and FLAIR images. MRA revealed stenotic changes of the bilateral middle cerebral artery (MCA), posterior cerebral arteries (PCA) and basilar artery (BA). Spinal MRI revealed hyperintense lesions within the cord extending from the T4 to the T6 level on the T2-weighted image. The patient fulfilled the clinical criteria of primary SS. In addition, anti-AQP4 antibody which is highly specific for NMO was detected in the serum at the acute phase. The patient excellently responded to IVIg while methylprednisolon pulse therapy was not effective. Follow-up MRA displayed complete resolution of the stenosis of the MCA, PCA and BA.
authors.affiliation: Department of Neurology, Mie University Graduate School of Medicine, 2-174, Edobashi, Tsu, Mie 514-8507, Japan. ii-y@clin.medic.mie-u.ac.jp
authors.forename: Yuichiro
authors.initials: Y
authors.lastname: Ii
authors.forename: Akihiro
authors.initials: A
authors.lastname: Shindo
authors.forename: Ryogen
authors.initials: R
authors.lastname: Sasaki
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Naito
authors.forename: Keiko
authors.initials: K
authors.lastname: Tanaka
authors.forename: Shigeki
authors.initials: S
authors.lastname: Kuzuhara
mesh_terms: D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D001157:Arterial Occlusive Diseases / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D001488:Basilar Artery / Q000473:pathology; D002539:Cerebral Arterial Diseases / Q000188:drug therapy; D002536:Cerebral Arteries / Q000473:pathology*; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D018810:Magnetic Resonance Angiography; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D009471:Neuromyelitis Optica / Q000150:complications* / Q000188:drug therapy; D020551:Pulse Therapy, Drug; D012859:Sjogren's Syndrome / Q000150:complications* / Q000188:drug therapy
chemical_list: D000893:Anti-Inflammatory Agents; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D008775:Methylprednisolone