特发性肥厚性硬脑膜炎临床、实验室检查及影像学特点分析

Clinical features, laboratory examination, and imaging features of idiopathic hypertrophic pachymeningitis: An analysis of 6 cases

摘要:

目的 分析6例特发性肥厚性硬脑膜炎（IHP）患者的临床特点及辅助检查特征。以提高对特发性肥厚性硬脑膜炎的认识。方法 回顾性分析并总结2014年1月至2017年11月在河南省人民医院神经内科就诊的6例临床诊断为特发性肥厚性硬脑膜炎（IHP）患者的临床、实验室检查结果及影像学特点。结果 6例患者主要临床表现为头痛、颅神经受累、癫痫、精神行为异常和共济失调。实验室检查血沉（ESR）、C反应蛋白（CRP）及脑脊液细胞和蛋白等炎性指标增高。磁共振影像表现为对称或不对称的硬脑膜T1加权等和（或）低信号，T2加权低信号，增强有强化。所有患者均给予激素冲击治疗，预后良好。结论 IHP以头痛及颅神经受累为主要表现，实验室检查炎性指标的升高及影像学硬脑膜增厚强化均有助于临床医师诊断及识别本病。

Abstract:

Objective To investigate the clinical features and auxiliary examination results of six patients with idiopathic hypertrophic pachymeningitis (IHP), and to raise the awareness of this disease.Methods A retrospective analysis was performed for the clinical data, laboratory results, and imaging findings of 6 patients who visited Department of Neurology in Henan Provincial People's Hospital from January 2014 to November 2017 and were diagnosed with IHP.Results The main clinical manifestations in these six patients were headache, cranial nerve involvement, epilepsy, mental and behavioral disorders, and ataxia. Laboratory examination showed increases in inflammatory indicators such as erythrocyte sedimentation rate, C-reactive protein, and cells and proteins in cerebrospinal fluid. Magnetic resonance imaging (MRI) showed symmetric or asymmetric isointensity and/or hypointensity on T1 weighted imaging and hypointensity on T2 weighted imaging of the cerebral dura mater, and contrast-enhanced MRI showed enhancement. All patients were given hormone shock therapy and achieved a good outcome.Conclusions Headache and cranial nerve involvement are the main manifestations of IHP. Increases in inflammatory indicators shown by laboratory examination and thickening and enhancement of the cerebral dura mater on imaging examination help with the diagnosis and identification of this disease.