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Bilateral Vision Loss Secondary to Pachymeningitis in a Patient with IgG4-Related Disease

Lucas Ramirez1, Andrea D'Auria1, Adeel Popalzai1

  • 1Roxanna Todd Hodges Comprehensive Stroke Clinic, University of Southern California , Los Angeles, CA , USA.

Frontiers in Neurology
|October 30, 2014
PubMed

Insights

Immunoglobulin G4-related disease (IgG4-RD) can affect the meninges, causing inflammation and thickening of the dura mater. This case highlights IgG4-RD as a cause of vision loss due to meningeal involvement.

Area of Science:

  • Neurology
  • Immunology
  • Ophthalmology

Background:

  • Immunoglobulin G4-related disease (IgG4-RD) is a multi-system fibroinflammatory condition.
  • Meningeal involvement in IgG4-RD can mimic other conditions like idiopathic hypertrophic pachymeningitis.
  • IgG4-RD can lead to significant organ damage and dysfunction.

Purpose of the Study:

  • To present a case of severe vision loss secondary to meningeal IgG4-related disease.
  • To highlight the diagnostic challenges and importance of considering IgG4-RD in cases of pachymeningitis.

Main Methods:

  • Clinical presentation of a patient with bilateral vision loss.
  • Magnetic Resonance Imaging (MRI) demonstrating dural thickening.
  • Histopathological examination of a dural biopsy for IgG4-related disease confirmation.

Main Results:

  • The patient experienced severe bilateral vision loss.
  • MRI revealed significant thickening of the dura mater.
  • Dural biopsy confirmed the diagnosis of IgG4-related disease.

Conclusions:

  • Meningeal IgG4-related disease is a potential cause of severe vision loss.
  • Early diagnosis and treatment of IgG4-RD are crucial to prevent irreversible complications.
  • This case underscores the importance of considering IgG4-RD in the differential diagnosis of hypertrophic pachymeningitis.
Keywords:
IgG4IgG4-related diseasedural thickeningpachymeningitisvision loss

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