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Isolated IgG4 hypertrophic pachymeningitis with cranial nerve involvement
Péter Faragó1, Tamás Zsigmond Kincses2, László Kovács3
1Department of Neurology, Albert Szent-Györgyi Clinical Center, University of Szeged, Szeged.
Ideggyogyaszati Szemle
|March 9, 2023
Summary
Immunoglobulin G4-related disease (IgG4-RD) is a rare autoimmune condition. This report details a rare case of IgG4-RD presenting as meningeal inflammation and cranial nerve involvement in an elderly male.
Area of Science:
- Neurology
- Immunology
- Rheumatology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a chronic, fibrotic autoimmune disorder.
- While often systemic, IgG4-RD can manifest in isolated organ involvement.
- Understanding rare presentations is crucial for timely diagnosis and treatment.
Observation:
- A case study of an elderly male patient with IgG4-related disease.
- The patient presented with diffuse meningeal inflammation and hypertrophic pachymeningitis.
- Neurological involvement included unilateral cranial nerve deficits and intraventricular abnormalities.
Findings:
- The case highlights a rare neurological manifestation of IgG4-RD.
- Diffuse meningeal enhancement and hypertrophic pachymeningitis were key imaging findings.
- Cranial nerve palsies and intraventricular extension indicate significant central nervous system impact.
Implications:
- This case expands the spectrum of known IgG4-RD presentations.
- Highlights the importance of considering IgG4-RD in elderly patients with unexplained meningeal inflammation.
- Suggests the need for specific diagnostic workup, including IgG4 levels and biopsies, in such cases.
