Rhombencephalitis as an initial manifestation of primary Sjögren's syndrome: a case report and review of the literatures.
OBJECTIVE
We present a case report and a comprehensive review of the literature concerning aseptic meningoencephalitis and Sjögren's syndrome (SS).


METHODS
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.


CONCLUSIONS
Primary 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 demonstrating brain lesions and in evaluating treatment efficacy of the SS.
Department of Neurology, National Taiwan University Hospital.
San-Feng
SF
Chen
Ming-Jang
MJ
Chiu
Jen-Jen
JJ
Su
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging / Q000379:methods; D008526:Medulla Oblongata / Q000473:pathology; D008590:Meningoencephalitis / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology*; D013902:Radiography, Thoracic; D012859:Sjogren's Syndrome / Q000503:physiopathology*; D013909:Thorax / Q000473:pathology; D014057:Tomography, X-Ray Computed / Q000379:methods
D000305:Adrenal Cortex Hormones; D000974:Antibodies, Antinuclear; D007166:Immunosuppressive Agents; C035356:SS-A antibodies; D003520:Cyclophosphamide