Treatment of myelopathy in Sjogren syndrome with a combination of prednisone and cyclophosphamide

Background: Peripheral neuropathy is a common complication of primary Sjogr en syndrome, but central nervous system involvement also occurs and may be the only extraglandular manifestation Sicca symptoms may also be minimal. C ombinations of lesions along with relapses and remissions can suggest multi ple sclerosis in the proper clinical setting, making the correct diagnosis elusive. Objectives: To report a case of progressive transverse myelopathy with prev ious optic neuropathy in primary central nervous system Sjogren syndrome (C NS-SS), and to review 17 previously reported cases and the patient's respon ses to various therapies. Design: Case report and literature review. Setting: University hospital. Patient: A 63-year-old Hispanic woman with a 10-month history of progressiv e spastic paraparesis associated with optic neuropathy and a T10 sensory le vel. Magnetic resonance imaging demonstrated multifocal, contrast-enhancing lesions in the spinal cord. The patient was diagnosed as having CNS-SS bec ause of the presence of sicca symptoms, abnormal serological test results, and salivary gland biopsy results, which fulfilled San Diego criteria for " definite" Sjogren syndrome. She responded to treatment with a combination o f prednisone and cyclophosphamide. Conclusions: Diagnosis of primary CNS-SS requires a high index of suspicion and specialized clinical testing. Treatment with pulse doses of corticoste roids alone may be suboptimal, but results of treatment with a combination of corticosteroids and either cyclophosphamide or chlorambucil have been en couraging.