IgG4-Related Hypertrophic Pachymeningitis Presenting with Features of Intracranial Hypertension: A Rare Case Report

Abstract

Background:

IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder that can involve virtually any organ system. Hypertrophic pachymeningitis is a rare neurological manifestation characterized by localized or diffuse dural thickening and enhancement. Early diagnosis is essential because the disease responds well to immunosuppressive therapy.

Case Presentation:

We report the case of a 40-year-old male who presented with symptoms suggestive of raised intracranial pressure. Magnetic resonance imaging (MRI) of the brain revealed diffuse pachymeningeal thickening and enhancement involving the cerebral convexities, falx cerebri, tentorium, and cavernous sinus regions. Initial laboratory evaluation demonstrated elevated serum IgG4 levels (approximately 5 g/L). Extensive investigations excluded infectious, neoplastic, and other autoimmune etiologies. Based on the clinical, radiological, and serological findings, a diagnosis of IgG4-related hypertrophic pachymeningitis was established.

Management and Outcome:

The patient was treated with corticosteroid therapy, resulting in significant clinical improvement and normalization of serum IgG4 levels on follow-up.

Conclusion:

 IgG4-related hypertrophic pachymeningitis should be considered in patients presenting with unexplained dural thickening and features of intracranial hypertension. Prompt diagnosis and treatment with corticosteroids can result in excellent clinical outcomes.