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Clivus-involved immunoglobulin G4 related hypertrophic pachymeningitis mimicking meningioma: A case report.
| Content Provider | Europe PMC |
|---|---|
| Author | Yu, Yang Lv, Liang Yin, Sen-Lin Chen, Cheng Jiang, Shu Zhou, Pei-Zhi |
| Copyright Year | 2022 |
| Abstract | BACKGROUNDImmunoglobulin G4 related disease (IgG4-RD) is a fibroinflammatory disease with markedly elevated serum IgG4 levels and fibrous tissue proliferation, accompanied by numerous plasma cells. IgG4 related hypertrophic pachymeningitis (IgG4-RHP) is relatively rare and indistinguishable from other phymatoid diseases before the operation. The risk of long-term immunosuppression needs to be balanced with disease activity. CASE SUMMARYA 40-year-old man presented with headache and bilateral abducent paralysis. He was also diagnosed with pulmonary tuberculosis 10 years ago and was on regular treatment for the same. Before the operation and steroid therapy, the patient was suspected of having tubercular meningitis at a local hospital. A clivus lesion was found via brain magnetic resonance imaging (MRI) at this presentation. He was preliminarily diagnosed with meningioma and underwent Gamma Knife Surgery. Transnasal endoscopic resection was performed to treat deterioration of nerve function. Postoperative pathologic examination suggested IgG4-RD. Moreover, the serum IgG4 was elevated at 1.90 g/L (reference range: 0.035-1.500 g/L). After steroid therapy for 2 mo, the lesion size diminished on MRI, and the function of bilateral abducent nerves recovered.CONCLUSIONIgG4-RHP is relatively rare and indistinguishable before the operation. Elevated serum IgG4 levels and imaging examination help in the diagnosis of IgG4-RHP. Surgery is necessary when lesions progress and patients start to develop cranial nerve function deficit. |
| Volume Number | 10 |
| PubMed Central reference number | PMC9254204 |
| Issue Number | 18 |
| PubMed reference number | 35949844 |
| Journal | World Journal of Clinical Cases |
| e-ISSN | 23078960 |
| DOI | 10.12998/wjcc.v10.i18.6269 |
| Language | English |
| Publisher | Baishideng Publishing Group Inc |
| Publisher Date | 2022-06-01 |
| Access Restriction | Open |
| Rights License | This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/ ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved. |
| Subject Keyword | Immunoglobulin G4 related disease Hypertrophic pachymeningitis Immunoglobulin G4 related hypertrophic pachymeningitis Clivus Case report |
| Content Type | Text |
| Resource Type | Article |
| Subject | Medicine |