肥厚性硬脑膜炎的临床分析

Clinical Presentations and Imaging Features of Hypertrophic Cranial Pachymengitis

目的:探讨肥厚性硬脑膜炎(HCP)的临床表现及影像学特征。方法:对我院确诊的1例及国内文献报道的153例HCP患者的临床资料进行回顾性分析。结果:154例HCP患者中,慢性起病138例(89.6%),首发症状为头痛者136例(88.3%)。头颅MRI检查主要见大脑镰、小脑幕及额、颞、顶部硬脑膜局限性或弥漫性增生肥厚,增强扫描可明显强化。73例(47.4%)HCP患者早期被误诊。128例应用皮质类固醇激素,11例激素减量过程中病情复发加用免疫抑制剂。结论:HCP主要以慢性头痛为首发症状,继而出现多组颅神经受损及小脑性共济失调等表现,头颅MRI增强扫描对本病诊断有重要价值,主要采用激素加免疫抑制剂治疗。

Objective： To explore the clinical presentations and imaging features of hypertrophic cranial pachymengitis（HCP）.Methods： The clinical data of one HCP patient diagnosed in our department and other 153 patients from literatures were analyzed retrospectively.Results： It was chronic onset in 138（89.6%）patients of 154 patients.The first sympotom of HCP was headache in 136（88.3%） patients.MRI of head demonstrated local or diffused thickened in cerebral flax,entorium of cerebellum,and the dura of frontal region,temporalis,parietalis and occiput,which could be enhanced through reinforced scanning.Seventy-three（47.4%） patients were misdiagnosed early.The 128 patients were treated by corticosteroid hormone and 11 patients whose pathogenetic condition recured were treated by immunodepressant.Conclusion： The first sympotom of HCP was chronic headache.It was multiple cranial nerves impariment and cerebellar ataxia subsequently.The MRI of head has an important significance for diagnosis.The treatments are corticosteroid hormone and immunodepressant