肥厚性硬脑膜炎13例临床及影像学分析

Clinical and magnetic resonance imaging analysis of 13 patients with hypertrophic cranial pachymeningitis

目的 探讨肥厚性硬脑膜炎的临床表现、辅助检查及治疗等,以提高对本病的认识.方法 回顾性分析2007年1月至2013年1月就诊于华山医院神经内科的13例经临床诊断及影像学证实的肥厚性硬脑膜炎患者.结果 13例患者均慢性起病,平均病程26.7个月（1.5个月～10年）,临床以慢性头痛（12/13）及颅神经麻痹症状（12/13）为主要表现.实验室检查示有非特异性炎症指标升高（10/13）,脑脊液蛋白升高（8/13）,均随治疗渐趋于正常.其中有2例患者合并血管炎指标异常,均表现为核周型抗中性粒细胞胞浆抗体（p-ANCA）阳性.所有患者脑脊液中均未发现病原体或肿瘤细胞.头颅磁共振成像（MRI）可见到小脑幕（10/13）、大脑镰（5/13）及额（4/13）、颞（7/13）、顶（4/13）硬脑膜局限性或弥漫性肥厚,在T1加权上表现为等信号,T2加权上表现为等信号或低信号,增强可见硬脑膜异常强化.多数患者经类固醇激素冲击治疗后症状明显好转（12/13）,1例患者用药过程中头痛症状有反复,在联合免疫抑制剂及放射治疗后,患者症状缓解.结论 肥厚性硬脑膜炎常表现为慢性头痛及颅神经麻痹,增强MRI可见特征性表现.类固醇激素冲击治疗能迅速缓解患者病情,是目前常用的治疗方法,但对于难治性肥厚性硬脑膜炎的患者,仍需考虑联合免疫抑制剂或放射治疗等.

Objective To investigate clinical presentations,laboratory examinations,magnetic resonance imaging （MRI） appearances and treatment of hypertrophic cranial pachymeningitis （HCP）.Methods The clinical data of 13 patients with HCP receiving comprehensive therapy in Huashan Hospital from January 2007 to January 2013 were analyzed retrospectively.Results The onset of HCP was mostly chronic with an average duration of 26.7 months.The main clinical manifestations of the 13 patients were chronic headaches （12/13） and cranial nerve paralysis （12/13）.Inflammation markers and cerebro-spinal fluid （CSF） protein levels increased in patients with HCP and gradually became normal after the treatment.The MRI demonstrated local or diffused thickened dura located in tentorium （10/13）,falx cerebrum （5/13）,frontal lobe （4/13）,temporal lobe （7/13） and parietal lobe （4/13）.The signal intensity was isointense on T1-weighted MR images and hypointense on T2-weighted MR images.Enhanced MR images showed conspicuous enhancement of the dural edges.Corticosteroid therapy improved the clinical symptoms in 12 of 13 patients.Conclusions HCP typically causes headache and paralysis of multiple cranial nerves.Enhanced MRI shows characteristic manifestations.At present corticosteroid therapy is the treatment of choice followed by immunosuppressive agent and radiotherapy.