Neuro-surgical considerations for treating IgG4-related disease with rare spinal epidural compression

Abstract

Background: Immunoglobulin G4-related disease (IgG4-RD) is a group of distinct autoimmune disorders affecting nearly every organ system in the body. Although central nervous system involvement is quite rare, it may present as hypertrophic pachymeningitis more frequently affecting the brain than the spine. In this study, we provide a case of spinal IgG4-RD pseudotumor resulting in cord compression, and a comprehensive review of the literature. Case Description: A patient presented with an extradural mass causing spinal cord compression at the L2-L3 level. Pathologically this proved to be an IgG4-RD pseudotumor. The patient was treated with thecal sac decompression and post-operative steroids that resulted in complete resolution of his symptoms. Conclusion: IgG4-RD is typically under-recognized and under-reported in the spinal literature. The clinical spinal presentation and non-surgical vs. surgical treatment are relatively straightforward. Although most cases can be managed with a course of steroids, surgical decompression may be required in patients presenting with spinal cord and/or nerve root compression. The differential diagnoses for these spinal tumors or pseudotumors should include IgG4-RD. Early detection and appropriate treatment can lead to satisfactory outcomes.