肥厚性硬膜炎的临床与影像学特点分析

Analysis for the clinical and imaging features of hypertrophic pachymeningitis

目的总结分析肥厚性硬膜炎的临床特点及影像学特征。方法回顾性分析2005年11月~2012年8月来收治的肥厚性硬膜炎10例临床资料。结果 10例患者中7例为HCP(hypertrophic cranial pachymeningtitis,肥厚性硬脑膜炎),1例为HSP(hypertrophic spinal pachymeningtitis,肥厚性硬脊膜炎),2例为HP(hypertrophic pachymeningitis,肥厚性硬膜炎)。男性7例、女性3例,年龄40~78岁,均为慢性起病,1例伴有系统性血管炎,1例患者20 y前曾行额窦骨瘤清除术,2例伴有双侧乳突、中耳炎,1例伴双侧乳突炎及上颌窦炎。7例HCP与2例HP患者均有头痛,其中2例伴有发热、1例患者出现抽搐、7例伴有脑神经受累、1例患者有共济失调;该例HSP患者症状为双下肢麻木无力,二便急,性功能下降。对9例患者行腰穿检查,其中蛋白增高者6例,细胞数增高者4例。对7例HCP与2例HP患者行颅脑与颈髓MRI平扫+增强检查,均见硬脑膜强化,小脑幕、大脑镰、颅底、颞部、鞍上池、小脑蚓等部位均可受累,其中以大脑镰、小脑幕强化最为明显,2例并存颈椎管内硬脊膜增厚强化,1例HSP行腰椎增强MRI,可见L5-S1硬脊膜增厚强化。对7例HCP与2例HP患者给予激素治疗1 w后头痛与脑神经麻痹症状明显好转,1例HSP患者接受激素治疗后脊髓受压症状及尿便障碍稍有好转。结论 HCP与HP患者主要表现为头痛及脑神经麻痹,HSP患者主要表现为神经根痛及脊髓受压症状。头部及脊髓MRI检查具有特征性,激素治疗临床效果满意。

Objective To explore the clinical and imaging features of hypertrophic pachymeningitis（ HP）. Methods The clinical data and MRI findings of 10 patients with HP admitted in our department from November 2005 were analyzed retrospectively. Results The 10 patients comprised 7 HCP,1 HSP,and 2HP. There were 7 male and 3 female.Their age ranged from 40 to 78 years old. All patients were chronic onset,1 case accompanied with systemic vasculltis,1case with a history of the removal operation of osteoma of frontal Sinus,2 cases accompanied with chronic mastoiditis and otitis media,1 case accompanied with mastoiditis and maxillary sinusitis. The 7 HCP and 2 HP all presented headache,2 cases with fever,1 case with epilepsy,7 cases with cranialnerve palsy,and 1 case with cerebellar ataxia. The HSP presented numbness and weakness of the lower limbs,ladder and rectal irritation symptoms,sexual dysfunction. Lumbar puncture of 9cases revealed that protein increased in 6 cases and cell increased in 4 cases. Enhanced MRI of head in 7 HCP and 2 HP demonstrated local or diffused thickening in dura mater,particularly in cerebral flax and entorium of cerebellum,enhanced MRI of lumbar vertebra in 1 case showed thickening in L5-S1 spinal dura mater. All 10 cases were treated by corticosteroid hormone,and their symptoms were relieved. Conclusion The main symptoms of HCP and HP are headache and cranial nerve palsy,the main symptoms of HSP are radiculalgia and symptoms of spinal cord compression. The MRI is the specificity examination. Corticosteroid hormone is effective for the treatment of HP.