肥厚性硬脑膜炎8例临床分析

Clinical features,neuroimaging findings and treatment ontcomes for eight cases with hypertrophic cranial pachymeningitis

目的总结分析肥厚性硬脑膜炎的临床特点及影像学特征，探讨其可能的发病机制。方法收集宣武医院2007年1月-2009年6月肥厚性硬脑膜炎8例临床资料进行分析。结果均慢性起病，头痛6例；多脑神经损害6例，其中动眼神经6例，舌咽、迷走、听神经各3例，视神经、外展神经各2例，舌下神经1例；癫痫发作2例。脑脊液：压力升高6例，白细胞数升高2例，蛋白升高8例，免疫球蛋白升高6例，白细胞数升高2例。血沉升高4例。血免疫球蛋白升高3例。血补体升高2例。头部MRI病变在T1、T2加权像呈等信号或低信号，增强扫描弥漫强化7例，局部强化1例，受累硬脑膜小脑幕5例，大脑镰、额顶部、海绵窦各3例，伴硬脊膜强化2例，继发静脉窦狭窄2例，无颅底强化病例。激素治疗7例疗效良好，其中1例持续高颅压手术治疗好转，1例未治疗好转；继发于乳突炎、中耳炎或鼻窦炎4例，特发性4例。结论肥厚性硬脑膜炎可发生于任何年龄。主要临床表现和头部MRI检查具有特征性，必要时活检确诊。皮质激素治疗效果好，必要时手术减压。

Objective To investigate clinical features, neuroimaging findings and treatment outcomes for patients with hypertrophic cranial pachymeningitis（HCP）. Methods 8 retrospective cases of HCP（6 male and 2 female） ,with a mean age of 54 years（ range from 21 to 74 years）were diagnosed through imaging studies, meningeal or orbital biopsy. The clinical features,neuroimaging findings and treatment outcome were documented for each case. Results The main clinical features were headache （ 6 cases）, cranial nerve palsy （ 6 cases ） and seizures （ 2 cases ）. The causes were idiopathic （ 2 ca ses）, mastorditis, tympanitis or nasosinusitis （ 4 cases）, and chronic granuloma inflammation （ 1 cases ）. Cerebrospinal fluid had increased pressure （ 6 cases）, elevated protein （ 8 cases ） and white cells （ 2 cases）. Erythrocyte sedimentation rates were elevated in 4 cases. The serum level of immunoglohulin （3 cases）and complement（ 2 cases）were high. On the initial MRI, the location of abnormal enhancement of the dura mater was associated with the clinical features. Corticosteroid therapy im proved the condition in 7 cases. 1 case of high CSF pressure get better by operation and 1 case released without treatment. Conclusion HCP is characterized by typical clinical features, neuroimaging findings and good response for corticosteroid therapy which must be defined by biopsy of the dura mater.