A selected case series of idiopathic hypertrophic pachymeningitis in a single center: Pathological characteristics and case-oriented review

J Neuroimmunol. 2023 Oct 15:383:578191. doi: 10.1016/j.jneuroim.2023.578191. Epub 2023 Aug 28.

Abstract

Hypertrophic pachymeningitis (HP) is a relatively rare disease of the central nervous system characterized by local or diffuse fibrous thickening of the dura mater. At present, there is still insufficient research on the pathogenesis and treatment strategies of this disease. We reported a continuous case series of seven patients with idiopathic HP (IHP), and also details one case of immunoglobulin G4-related HP requiring surgical intervention. Early diagnosis and appropriate surgical intervention for IHP could prevent the progression of permanent neurological damage and spinal cord paraplegia.

Keywords: Hypertrophic pachymeningitis; Immune disease; Steroid therapy; Surgical intervention.

Publication types

  • Case Reports
  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Dura Mater / diagnostic imaging
  • Dura Mater / pathology
  • Dura Mater / surgery
  • Humans
  • Hypertrophy
  • Meningitis* / complications
  • Meningitis* / diagnostic imaging
  • Spinal Cord / pathology