Objective To summarize the characteristics of IgG4-related hypertrophic pachymeningitis (IgG-RHP) from clinical , imaging and pathological aspects by a retrospective study. Methods From January 2014 to December 2016 , the data of the inpatients with IgG4-RHP in Beijing Tiantan Hospital were collected , and their clinical manifestation , imaging and pathological characteristics were analyzed by using a descriptive method. Results There were 6 patients ,2 males and 4 females. The age ranges from 27 years old to 71 years old.The most common clinical symptoms were :chronic headache (6/6) ,injury of cranial nerves (3/6) and seizures (2/6) . The serum IgG4 levels of all the patients were higher than the normal upper limit. Intracranial abnormal signal was seen in magnetic resonance imaging (MRI) . Cranial magnetic resonance imaging showed dural enhancement. Brain biopsy was performed in 6 patients and the pathologic results were consistent with IgG4-RHP.Conclusions IgG4-RHP is not uncommon. Serum levels of IgG4 should be further detected in patients with hypertrophic meningitis. For early diagnosis and early treatment , dura biopsy is sometimes required.%目的 总结IgG4相关肥厚性硬脑膜炎(IgG4-related hypertrophic pachymeningitis,IgG4-RHP)的临床、M RI和病理学特点.方法 收集2014-01-01—2016-12-31首都医科大学附属北京天坛医院神经病学中心神经感染与免疫科收治的IgG4-RHP患者的临床资料,分析其临床表现、MRI及病理学特点.结果 共收集6例患者,其中男2例,女4例,首发症状主要为为慢性头痛6例、脑神经损伤3例、痫性发作2例.所有患者血清IgG4水平均升高,头颅MRI检查均可见硬脑膜强化.所有患者病理学检查结果符合IgG4-RHP表现.结论IgG4-RHP在临床中并不少见,对于诊断肥厚性硬脑膜炎的患者应进一步行血清IgG4水平检测,必要时行硬脑膜活检以助于早诊断、早治疗.
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