Abstract: Monoclonal immunoglobulin disease is comprised of a group of heterogeneous diseases，which is characterized by the presence of monoclonal immunoglobulin in blood or urine. The clinical manifestations of the disease are versatile，and neurological dysfunction is one of the clinical manifestations.  In our hospital, a patient with monoclonal immunoglobulinemia complicated with neurological dysfunction was diagnosed as BingNeel syndrome, given RFC regimen chemotherapy（rituximab 600 mg d1+flumurabin 40 mg d24+cyclophosphamide 0.3  g d24）, combined with  intrathecal injection, the patient's  clinical symptoms improved significantly.

Key words: monoclonal gammopathies；abnormal neurological function；diagnosis, treatment