Abstract: Objective　To explore the clinical features of patients with hypertrophic perimeningitis and increased serum IgG4. Methods　Twenty patients with hypertrophic pachymeningitis and increased serum IgG4 who were hospitalized in Guangdong 999 Brain Hospital from January 2018 to January 2021 were collected, and their clinical data were retrospectively analyzed. Results　Among the 20 patients, there were 9 females and 11 males. The age of onset was (50.8±16.5). Their main clinical manifestations were headache [90.0% (18/20)], decreased vision [35.0% (7/20)], diplopia [25.0% (5/20)], tinnitus [25.0% (5/20)], fever [20.0% (4/20)], epilepsy [20.0% (4/20)], drooping eyelids [15.0% (3/20)], and limb weakness 10.0% (2/20). The enhancement of cranial MR in all the patients indicated that the dura mater was strengthened and the serum level of IgG4 was elevated. Conclusions　Hypertrophic pachymeningitis with elevated serum IgG4 has various MRI manifestations, mainly involving the dura mater, and could be combined with skull destruction and cerebral cortex infiltration damage. All patients considering the diagnosis of hypertrophic pachymeningitis should detect serum IgG4, and take meningeal biopsy if it is necessary to further confirm the diagnosis.

Key words: Meningitis, IgG4-related hypertrophic pachymeningitis, Magnetic resonance imaging, Clinical features, IgG4-related diseases

摘要： 目的　探索以血清免疫球蛋白（Ig）G4水平升高的肥厚性硬脑膜炎患者的临床特征。方法　收集2018年1月至2021年1月在广东三九脑科医院住院治疗的20例血清IgG4水平升高的肥厚性硬脑膜炎患者并回顾性分析其临床资料。结果　20例患者中，女9例、男11例，发病年龄（50.8±16.5）岁。患者的主要临床表现为头痛［90.0%（18/20）］、视力下降［35.0%（7/20）］、视物重影［25.0%（5/20）］、耳鸣［25.0%（5/20）］、发热［20.0%（4/20）］、癫痫［20.0%（4/20）］、眼睑下垂［15.0%（3/20）］、肢体无力［10.0%（2/20）］。所有患者头颅MR增强提示硬脑膜强化且血清中IgG4水平升高。结论　以血清中IgG4升高的肥厚性硬脑膜炎在磁共振成像的表现主要以累及硬脑膜为主，可有颅骨破坏及大脑皮层浸润性损害。在临床怀疑为肥厚性硬脑膜炎患者，建议完善血清IgG4检测，必要时可进一步病理学活检以明确诊断。

关键词: 脑膜炎, IgG4相关性肥厚性脑膜炎, 磁共振成像, 临床特点, IgG4相关性疾病