title: Efficacy of extracranial-intracranial bypass for progressive middle cerebral artery occlusion associated with active Sjögren's syndrome: case report.
abstract: Sjögren syndrome affecting the major cerebral arteries is rare, and an optimal therapeutic strategy to counteract such a lesion has not yet been established. We herein report a case of a 39-year-old woman with a history of primary Sjögren syndrome, which had previously been treated with immunosuppressive therapy, manifesting with a crescendo transient ischemic attack because of left middle cerebral artery stenosis. Despite the administration of high doses of prednisolone and azathioprine for active Sjögren syndrome, the frequency of crescendo transient ischemic attacks increased with the progression of stenosis and magnetic resonance imaging showed the development of subacute cerebral infarction. Single-photon emission computed tomography with N-isopropyl[(123)I]-p-iodoamphetamine revealed apparent hemodynamic compromise in the affected cerebral hemisphere. In light of the increased risk of further progression of cerebral infarction, we decided to perform surgical revascularization in spite of her active inflammatory condition. The patient underwent extracranial-intracranial bypass without complications and was treated with intensive immunosuppressive therapy during the perioperative period. Based on our findings, we recommend surgical revascularization for occlusive cerebrovascular disease with hemodynamic compromise in combination with intensive immunosuppressive therapy, even in the active inflammatory state of autoimmune diseases, if ischemic symptoms are medically uncontrollable.
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Sakata
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Japan. Electronic address: fujimur@nsg.med.tohoku.ac.jp.
authors.forename: Miki
authors.initials: M
authors.lastname: Fujimura
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Sato
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Shimizu
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Teiji
authors.initials: T
authors.lastname: Tominaga
mesh_terms: D000328:Adult; D002544:Cerebral Infarction / Q000517:prevention & control*; D002548:Cerebral Revascularization / Q000379:methods*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D002546:Ischemic Attack, Transient / Q000150:complications / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D008279:Magnetic Resonance Imaging; D020768:Middle Cerebral Artery / Q000473:pathology*; D019060:Minimally Invasive Surgical Procedures; D019635:Neurosurgical Procedures / Q000379:methods; D012859:Sjogren's Syndrome / Q000150:complications* / Q000188:drug therapy; D015899:Tomography, Emission-Computed, Single-Photon; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents
keywords: Sjögren's syndrome; cerebral artery occlusion; cerebral infarction; immunosuppressive therapy