Rhombencephalitis as an initial manifestation of primary Sjögren's syndrome: A case report and review of the literatures
Journal
Acta Neurologica Taiwanica
Journal Volume
20
Journal Issue
1
Pages
35-41
Date Issued
2011
Author(s)
Abstract
Purpose: We present a case report and a comprehensive review of the literature concerning aseptic meningoencephalitis and Sj?gren's syndrome (SS). Case Report: We report a 44-year-old woman of primary SS with initial presentation of aseptic meningoencephalitis and a reversible magnetic resonance image (MRI) lesion in the medulla. The diagnosis of primary SS based on ocular dryness, lacrimal hyposecretion, secretory and excretory dysfunction from sialocintigraphy, and positive anti-SS-A antibodies. Corticosteroid and cyclophosphamide therapies reversed the neurological deficits and the MRI lesion. Conclusion: Primayr SS may have variable manifestations in the central nervous system which may precede the classic sicca symptoms. SS should be investigated in cases of aseptic meningoencephalitis even without clinical signs of xerostomia or xerophthalmia. MRI is useful in demostrating brain lesions and in evaluating treatment efficacy of the SS.
SDGs
Other Subjects
cyclophosphamide; hydroxychloroquine; methylprednisolone; Ro antibody; adult; antibody detection; article; balloon catheter; body temperature; case report; computer assisted tomography; corticosteroid therapy; diplopia; dizziness; drug dose reduction; dry eye; dysphagia; dyspnea; evoked somatosensory response; evoked visual response; female; human; hyperreflexia; interstitial lung disease; lacrimation disorder; limb weakness; lobar pneumonia; medulla oblongata; meningoencephalitis; muscle strength; nasogastric tube; nausea; nerve conduction; neurologic examination; nuclear magnetic resonance imaging; plasmapheresis; polyradiculoneuropathy; prick test; Schirmer test; scintigraphy; serology; Sjoegren syndrome; thorax radiography; tracheotomy; unsteadiness; vertigo; vomiting; Adrenal Cortex Hormones; Adult; Antibodies, Antinuclear; Cyclophosphamide; Female; Humans; Immunosuppressive Agents; Magnetic Resonance Imaging; Medulla Oblongata; Meningoencephalitis; Radiography, Thoracic; Sjogren's Syndrome; Thorax; Tomography, X-Ray Computed
Type
journal article