Central nervous system involvement in primary Sjögren's syndrome

Abstract

Clinical and imaging data of 4 cases of primary Sjögren's syndrome (pSS) combined with central nervous system (CNS) lesions were retrospectively analyzed. Clinical symptoms of 4 patients were as follows: 2 cases had fever; 2 cases had blurred vision; 2 cases had lower extremity weakness; one case had single lower limb weakness; one case had unilateral limb weakness; one case had hemisensory reduction; one case had bilateral abducens paralysis; 3 cases had peripheral neuropathy. Analysis of cerebrospinal fluid (CSF) revealed increased CSF pressure (210-270 mm H2O) in 3 cases, increased number of leukocyte [(50-380) × 10 6/L] in 3 cases, and increased protein (1.30-2.56 g/L) in 3 cases. Brain MRI showed multiple lesions in white matters. After high-dose steroid therapy, 3 cases had good prognosis, and one had recurrent lower extremity weakness after 6 months. pSS combined with CNS lesions is rare. Lumbar puncture and brain MRI is important for diagnosis, and high-dose steroid therapy may have good therapeutic effect.

DOI: 10.3969/j.issn.1672-6731.2015.04.014