Abstract:

Methods The clinical materials of a patient with CNSL complicated with SS were collected. The diagnosis process was summarized and the relevant literatures were reviewed. Results The female patient，51 year old，was admitted to the hospital for dry mouth and dry eyes with nausea and vomiting for more than one month.The physical examination results showed the tongue surface was dry，the patient moved restlessly at times，and the limb strength was level 4；the left calcaneal and tibial tests were not stable， and the Romberg’s sign were positive and bilateral pathological signs were positive.The results of laboratory tests showed anti-Sjogren’s syndrome A antibody（anti-SSA antibody） and anti-Sjogren’s syndrome B antibody（anti-SSB antibody） were positive.The results of tear secretion test showed left side 5 mm； the corneal staining was positive，and salivary gland isotope test was positive. The enhanced magnetic resonance imaging（MRI） results showed multiple abnormal enhancement signals in bilateral medial cerebellar hemispheres， optic chiasm， pituitary stalk and pineal gland；the magnetic resonance spectrum（MRS） results of the head revealed that the N-acetyl-L-aspartic acid（NAA） peak and creatine（Cr） peak of L-aspartic acid were decreased， while the choline（Cho） peak was increased， NAA/Cr=1.74， Cho/Cr=3.52， Cho/NAA=2.02；the lactate（Lac） peak and lipid（Lip） peak were also seen， single-voxel ¹H-MRS and multi-voxel ¹H-MRS were found in bilateral cerebellar hemispheres. The pathological examination showed that high-grade B-cell lymphoma with hemorrhage and the clinical diagnosis was CNSL complicated with SS. Radiotherapy and chemotherapy were given after discharge in local hospital. Conclusion The incidence of CNSL with SS is low， and it is easy to be misdiagnosed as central nervous system demyelinating disease. CNSL should be considered when the symptoms and signs of central nervous system are present in the SS patients. Objective To analyze the clinical manifestation， diagnosis and treatment process of a patient with central nervous system lymphoma （CNSL） complicated with Sj?gren’s syndrome（SS） and explore its pathogenesis， and to provide reference for the diagnosis of this rare disease.

Key words: Central nervous system lymphoma, Sjogren’s syndrome, B lymphocyte, Autoimmune disease