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Inflammatory Pseudotumor of the Brain Parenchyma with IgG4 Hypergammaglobulinemia

Haruko Tanji1, Hiroaki Okada, Ryosuke Igari

  • 1Department of Neurology, Hematology, Metabolism, Endocrinology and Diabetology (DNHMED), Yamagata University School of Medicine, Japan.

Internal Medicine (Tokyo, Japan)
|July 20, 2016
PubMed

Insights

This case study presents a rare instance of brain lesions with elevated immunoglobulin G4 (IgG4) levels, responsive to steroid treatment. Further research is needed to clarify IgG4

Area of Science:

  • Neurology
  • Immunology
  • Gastroenterology

Background:

  • A 58-year-old woman presented with neurological deficits including sensory aphasia and right hemiparesis.
  • Laboratory findings revealed elevated serum IgG and IgG4, pancytopenia, and liver dysfunction, with abdominal imaging suggestive of sclerosing cholangitis.

Observation:

  • Brain MRI demonstrated extensive signal abnormalities and an enhancing lesion in the left hemisphere.
  • Brain biopsy showed inflammatory infiltrates, predominantly IgG-positive plasma cells, with sparse IgG4-positive cells.

Findings:

  • The patient's neurological symptoms and MRI abnormalities significantly improved after betamethasone treatment.
  • Histopathological findings did not meet the diagnostic criteria for IgG4-related disease.

Implications:

  • This is the first reported case of tumefactive brain lesions with elevated serum IgG4 responsive to steroids.
  • More case reports with detailed pathological analysis are crucial for understanding the role of IgG4 in related disorders.

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