Immunoglobulin G4-related hypertrophic pachymeningitis with spinal cord compression: A case report

J Neuroimmunol. 2020 Oct 15:347:577325. doi: 10.1016/j.jneuroim.2020.577325. Epub 2020 Jul 14.

Abstract

IgG4-related disease (IgG4-RD) is a recently recognized inflammatory condition that can be found in many organs. However, spinal involvement is rare and has been described only in case reports and series. Here, we report a rare case of spinal IgG4-RD that resulted in hypertrophic pachymeningitis with spinal cord compression. This case expands the phenotypic presentation for the neurological sequelae of IgG4-RD. Our case hints that spinal IgG4-RD may be misdiagnosed, and IgG4-RD in patients should be considered when the patient has a dural mass. Although early surgery, steroids, and/or immunosuppressive therapy may prevent neurological complications, the side effects should receive more attention during treatment.

Keywords: Hypertrophic pachymeningitis; IgG4-related disease (IgG4-RD); Spinal cord compression.

Publication types

  • Case Reports

MeSH terms

  • Female
  • Humans
  • Immunoglobulin G4-Related Disease / complications*
  • Immunoglobulin G4-Related Disease / diagnostic imaging*
  • Immunoglobulin G4-Related Disease / surgery
  • Meningitis / complications*
  • Meningitis / diagnostic imaging*
  • Meningitis / surgery
  • Middle Aged
  • Spinal Cord Compression / complications*
  • Spinal Cord Compression / diagnostic imaging*
  • Spinal Cord Compression / surgery
  • Thoracic Vertebrae / diagnostic imaging
  • Thoracic Vertebrae / surgery