Immunoglobulin G4-related disease involving both cerebral parenchyma and spinal cord: A case report.

IgG4-related disease (IgG4-RD) is an immune-mediated condition that affects multiple organ systems with tumorlike swelling. Here, for the first time, we report a rare case of IgG4-RD that involves the cerebral parenchyma, intramedullary spinal cord and lymph nodes. The diagnosis of IgG4-RD was made based on histological features, multi organ involvement and high serum IgG4 levels. The patient responded well to glucocorticoid treatment. Our case broadens the phenotypic presentation for the neurological sequelae of IgG4-RD.