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Immune-Mediated Hypertrophic Pachymeningitis and its Mimickers: Magnetic Resonance Imaging Findings

Published:March 06, 2023DOI:https://doi.org/10.1016/j.acra.2023.01.017
      Hypertrophic pachymeningitis (HP) is a rare and chronic inflammatory disorder presenting as localized or diffuse thickening of the dura mater. It can be idiopathic or an unusual manifestation of immune-mediated, infectious, and neoplastic conditions. Although some cases may remain asymptomatic, HP can lead to progressive headaches, cranial nerve palsies, hydrocephalus, and other neurological complications, which makes its recognition a fundamental step for prompt treatment. Regarding the diagnosis workup, enhanced MRI is the most useful imaging method to evaluate dural thickening. This article addresses the MR imaging patterns of immune-mediated HP, including immunoglobulin G4-related disease, neurosarcoidosis, granulomatosis with polyangiitis, rheumatoid pachymeningitis, and idiopathic HP. The main infectious and neoplastic mimicking entities are also discussed with reference to conventional and advanced MR sequences.

      Graphical Abstract

      Key Words

      Abbreviations:

      HP (Hypertrophic Pachymeningitis), CT (Computed Tomography), MRI (Magnetic Resonance Imaging), WI (Weighted Imaging), IgG4-RD (Immunoglobulin G4-Related Disease), NS (Neurosarcoidosis), GPA (Granulomatosis with Polyangiitis), RP (Rheumatoid pachymeningitis), IH (Intracranial Hypotension), CNS (Central nervous system), PNS (Peripheral nervous system), RA (Rheumatoid Arthritis), CSF (Cerebrospinal fluid), VDRL (Venereal Disease Research Laboratory), FTA-ABS (Fluorescent Treponemal Antibody Absorption)
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