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A Case of IgG4-related Hypertrophic Pachymeningitis Kentaro HAYASHI 1 , Chika SOMAGAWA 1 , Yukishige HAYASHI 1 , Mitsuto IWANAGA 1 1Department of Neurosurgery, Sasebo City General Hospital Keyword: hypertrophic pachymeningitis , IgG4 related disease , IgG4 pp.1103-1109
Published Date 2018/12/10
DOI https://doi.org/10.11477/mf.1436203876
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 Hypertrophic pachymeningitis is a chronic inflammatory disease, manifesting as a fibrous thickening of the dura mater. Recently, it was reported that IgG4 is related to the etiology of some diseases, such as pancreatitis. Here, we report a case of hypertrophic pachymeningitis related to infiltration of IgG4 positive plasma cells. A 68-year-old man presented at a nearby hospital with a headache and a low-grade fever. A blood test revealed inflammation, as well as elevation of IgG4 level. Magnetic resonance imaging(MRI)revealed diffuse thickening of the dura mater, dominantly in the posterior fossa and cerebellar tentorium. The lesion was enhanced significantly with gadolinium(Gd). An open biopsy was performed to determine pathological diagnosis. Hematoxylin and eosin staining showed infiltration of inflammatory cells, including plasma cells. The infiltrating cells were positive for the IgG4. Post-operatively, the patient was treated with glucocorticoid, and both the inflammation and patient symptoms were improved. In conclusion, IgG4 is related to the etiology of hypertrophic pachymeningitis and glucocorticoid therapy is effective for this disease.


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電子版ISSN 1882-1251 印刷版ISSN 0301-2603 医学書院

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