The authors present a patient with Sjögren's syndrome with a fluctuating and then progressive myelopathic syndrome, and optic nerve involvement. Treatment with chlorambucil and prednisone improved the patient's function from being wheelchair bound to walking unaided. Spinal MRI showed multiple, extensive intraparenchymal areas of abnormal T2-weighted signal intensity, gadolinium enhancement, and cord swelling, which also improved during the period of treatment.