Abstract
Primary Sjögren syndrome, which involves lesions in both the brain and spinal cord, is rarely reported. Related symptoms, such as intractable pain due to central nervous system involvement, are very rare. A 73-year-old woman diagnosed with primary Sjögren syndrome manifested with subacute encephalopathy and extensive transverse myelitis. She complained of severe whole body neuropathic pain. Magnetic resonance imaging demonstrated a non-enhancing ill-defined high intensity signal involving the posterior limb of the both internal capsule and right thalamus on a T2 fluid-attenuated inversion recovery image. Additionally, multifocal intramedullary ill-defined contrast-enhancing lesion with cord swelling from the C-spine to L-spine was also visible on the T2-weighted image. Her intractable pain remarkably improved after administration of concomitant oral doses of gabapentin, venlafaxine, and carbamazepine.