特发性肥厚性硬脑膜炎5例临床分析并文献复习  

Clinical Analysis of Idiopathic Hypertrophic Pachymeningitis: 5 Case Reports and Literature Review

作　　者：孙金梅[1] 郑晓丹[2] 张成杰[1] 韩燕飞[1] 韩春玉[1] SUN Jin-mei;ZHENG Xiao-dan;ZHANG Cheng-jie;HAN Yan-fei;HAN Chun-yu(Department of Neurology,Capital Medical University,Beijing 100050,China;Department of Pathology,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China)

机构地区：[1]首都医科大学附属北京友谊医院神经内科,北京100050 [2]首都医科大学附属北京友谊医院病理科,北京100050

出　　处：《神经损伤与功能重建》2020年第3期151-154,共4页Neural Injury and Functional Reconstruction

基　　金：国家自然基金(No.81801334);首都医科大学附属北京友谊医院院启动课题(No.yyqdkt2017-13)。

摘　　要：目的:探讨特发性肥厚性硬脑膜炎(IHP)的临床特点。方法:回顾性分析5例IHP患者的临床资料、实验室检查、影像资料及治疗情况。结果:5例患者均为男性,中位年龄57岁,均以头痛为首发症状,5例脑脊液蛋白水平偏高,4例血沉结果异常,1例C-反应蛋白水平偏高,5例ANA结果均异常,5例头颅MRI+增强检查可见弥漫性或局灶性硬脑膜强化,2例经病理活检确诊。5例患者均接受大剂量激素冲击继予小剂量激素长期维持治疗后,头痛均基本缓解。随访期1年,失联1例,另4例头痛无复发。结论:IHP临床较少见,多以头痛为主要表现,同时可伴颅神经受损表现,该病患者血沉、C反应蛋白可升高,风湿免疫系列中ANA结果普遍异常,头颅MRI+增强是其诊断的重要手段,病理活检是确诊的金标准,预后较好,但有复发风险。Objective: To explore the clinical features of idiopathic hypertrophic pachymeningitis(IHP).Methods: Total 5 patients with IHP were enrolled. Their clinical profiles, laboratory examination, MRI features, and treatment were retrospectively analyzed. Results: All 5 patients were male. The median age was 57.All patients experienced headache as initial symptom. Cerebrospinal fluid protein level was elevated in all patients, and blood sedimentation was abnormal in 4 patients. C-reactive protein level was elevated in 1 patient.ANA results were abnormal in all patients. All patients showed diffuse or focal dura mater enhancement on MRI. Diagnosis of IHP was confirmed with biopsy in 2 patients. All received large dose hormones followed by small doses of hormones, and after extended treatment, headache was relieved in all patients. Patients were followed-up for 1 year during which contact was lost with 1 patient and the remaining 4 patients showed no recurrence of headache. Conclusion: IHP is clinically rare. Headache is the most common symptom, and it may also present with cranial neuropathy or other neurological deficits. Blood sedimentation and C-reactive protein in patients may be elevated, and rheumatoid immune series ANA results are usually abnormal. Abnormal signals on MRI provide important clues for diagnosis, and pathological biopsy is the gold standard for confirming diagnosis. Most patients show good prognosis, but there is a risk of recurrence.

关 键 词：特发性肥厚性硬脑膜炎 头痛 静脉窦血栓形成 

分 类 号：R741[医药卫生—神经病学与精神病学] R742.6[医药卫生—临床医学]