IgG4-Related Spinal Hypertrophic Pachymeningitis With Neurological Deficit: A Report of 2 Cases

JBJS Case Connect. 2023 Jun 1;13(2). doi: 10.2106/JBJS.CC.23.00024. eCollection 2023 Apr 1.

Abstract

Case: Immunoglobulin G4 (IgG4)-related spinal hypertrophic pachymeningitis (IgG4-RHP) is a rare disease characterized by diffuse inflammatory fibrosis of the spinal dura mater that can cause myelopathy and neurological deficits. Here, we report 2 cases in which both patients presented with myelopathy and paraplegia. They underwent decompressive laminectomy, and the diagnoses were confirmed through histopathologic examination. Both patients received prednisolone postoperatively, which was subsequently switched to methotrexate for maintenance.

Conclusion: Two cases of IgG4-RHP were successfully treated with decompressive laminectomy and combined therapy with steroids and other immunosuppressive agents.

Publication types

  • Case Reports

MeSH terms

  • Humans
  • Hypertrophy
  • Immunoglobulin G
  • Meningitis* / complications
  • Meningitis* / diagnosis
  • Meningitis* / pathology
  • Spinal Cord Diseases*
  • Spine / pathology

Substances

  • Immunoglobulin G