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KMID : 1201420160090020162
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Journal of Neurocritical Care
2016 Volume.9 No. 2 p.162 ~ p.165
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A Case of IgG4 Related Pachymeningitis
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Kim Ji-In
Song Jin-Taek
Kwon Hyeong-Ju
Lee Ji-Yong
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Abstract
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Background: Immunoglobulin G4 (IgG4) related disease is an immune-mediated fibroinflammatory condition that can affect multiple organs. There are only few reports of pachymeningeal involvement by IgG4 related disease in Korea.
Case Report: A 50-year-old woman was admitted due to generalized seizure and headache. Brain magnetic resonance imaging (MRI) showed nodular dural thickening and enhancement in the right frontal areas. A meningeal biopsy was performed. On pathology, chronic inflammation, fibrosis and plasma cell infiltration were revealed. Immunoglobulin G4 immunohistochemistry showed prominent IgG4+ plasma cell infiltrate. Serum IgG level was normal but IgG4 subclass level was significantly elevated. She was treated with intravenous dexamethasone followed by oral prednisolone. After 1 year of treatment, brain MRI showed marked decrease of dural thickening and enhancement.
Conclusion: We report a case of IgG4 related pachymeningitis improved with corticosteroid. IgG4 related disease should be included in the differential diagnosis in patients with hypertrophic pachymeningitis.
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KEYWORD
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Immunoglobulin G4, Pachymeningitis, Corticosteroid
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