Hayashi Kentaro, Somagawa Chika, Hayashi Yukishige, Iwanaga Mitsuto
Department of Neurosurgery, Sasebo City General Hospital.
No Shinkei Geka. 2018 Dec;46(12):1103-1109. doi: 10.11477/mf.1436203876.
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.
肥厚性硬脑膜炎是一种慢性炎症性疾病，表现为硬脑膜的纤维性增厚。最近，有报道称IgG4与某些疾病如胰腺炎的病因有关。在此，我们报告一例与IgG4阳性浆细胞浸润相关的肥厚性硬脑膜炎病例。一名68岁男性因头痛和低热到附近医院就诊。血液检查显示有炎症，IgG4水平也升高。磁共振成像（MRI）显示硬脑膜弥漫性增厚，主要位于后颅窝和小脑幕。病变在钆（Gd）增强后明显强化。进行了开放性活检以确定病理诊断。苏木精-伊红染色显示有包括浆细胞在内的炎性细胞浸润。浸润细胞IgG4呈阳性。术后，患者接受糖皮质激素治疗，炎症和患者症状均得到改善。总之，IgG4与肥厚性硬脑膜炎的病因有关，糖皮质激素治疗对该疾病有效。
