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Updated: Sep 21, 2025

Isolating Central Nervous System Tissues and Associated Meninges for the Downstream Analysis of Immune cells
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Isolating Central Nervous System Tissues and Associated Meninges for the Downstream Analysis of Immune cells

Published on: May 19, 2020

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[A case of IgG4-related disease exclusively affecting pia matter and cerebral parenchyma]

Shohei Beppu1, Taiki Yabumoto1, Makoto Kinoshita1

  • 1Department of Neurology, Osaka University Graduate School of Medicine.

Rinsho Shinkeigaku = Clinical Neurology
|June 1, 2022
PubMed

Insights

This case study shows that IgG4-related disease (IgG4-RD) can cause isolated brain lesions. Early diagnosis and treatment with steroids can significantly improve cognitive function and imaging results.

Area of Science:

  • Neurology
  • Immunology
  • Pathology

Background:

  • Immunoglobulin G4-related disease (IgG4-RD) is a rare fibroinflammatory condition.
  • It can affect various organs, but isolated central nervous system involvement is uncommon.

Observation:

  • A 61-year-old man presented with progressive cognitive decline and MRI-detected left frontal lobe lesions with leptomeningeal enhancement.
  • Histopathology confirmed IgG4-related disease (IgG4-RD).
  • Serum IgG4 levels were normal, and no other organ systems were clinically implicated.

Findings:

  • Despite normal serum IgG4 levels, the patient's presentation was consistent with IgG4-related disease (IgG4-RD).
  • Treatment with intravenous methylprednisolone and oral prednisone led to marked improvement in cognitive function and MRI findings.

Implications:

  • This case underscores the importance of considering IgG4-related disease (IgG4-RD) in the differential diagnosis of isolated cranial lesions.
  • It highlights that normal serum IgG4 levels do not exclude the diagnosis.
  • Prompt diagnosis and immunosuppressive therapy can lead to favorable neurological outcomes.
Keywords:
IgG4-related diseasebrain biopsycentral nervous system lesionscognitive decline

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