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NeuroImages: IgG4-related disease with meningeal and parenchymal involvement on MRI and histopathology
Lina Li1, Haibing Xiao2,3
1Department of Neuromedicine Center, The University of Hong Kong-Shenzhen Hospital, Shenzhen, Guangdong, China.
Abstract:
IgG4-related disease (IgG4-RD) rarely involves the CNS parenchyma. We report a 44-year-old man with left facial numbness and seizures. MRI showed right frontal-parietal dural thickening and parenchymal edema. Biopsy revealed IgG4 + plasma cells (> 50/HPF) with obliterative phlebitis, confirming IgG4-RD. This case highlights the need to consider IgG4-RD in meningo-parenchymal lesions mimicking tumors or infections. Glucocorticoids should be initiated promptly after histopathologic confirmation.
Insights
Immunoglobulin G4-related disease (IgG4-RD) is a rare condition affecting the central nervous system. This case highlights the importance of considering IgG4-RD in meningo-parenchymal lesions for prompt diagnosis and treatment.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition.
- Central Nervous System (CNS) parenchymal involvement in IgG4-RD is exceptionally rare.
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