幕下中枢神经系统表面铁沉积症三例报告并文献复习  

Infratentorial superficial siderosis of central nervous system: three cases report and literature review

作　　者：孙云闯[1] 李凡[1] 金海强[1] 王朝霞[1] 孙葳[1] SUN Yun-chuang;LI Fan;JIN Hai-qiang;WANG Zhao-xia;SUN Wei(Department of Neurology,Peking University First Hospital,Beijing 100034,China)

机构地区：[1]北京大学第一医院神经内科,100034

出　　处：《中国现代神经疾病杂志》2021年第10期893-898,共6页Chinese Journal of Contemporary Neurology and Neurosurgery

基　　金：“重大新药创制”科技重大专项项目(项目编号:2017ZX09304028)。

摘　　要：目的报告3例幕下中枢神经系统表面铁沉积症病例,分析其病因和临床表现。方法与结果临床症状主要表现为反复发作性头痛或癫(3例),伴听力明显减退(2例);腰椎穿刺脑脊液外观呈浑浊血性液体,红细胞计数增加、蛋白定量升高(2例);头部或颈椎MRI显示小脑半球、脑桥、中脑表面铁沉积,增强后病灶无强化表现(3例),同时可见颈静脉孔区占位性病变(例1)、颈髓表面血管迂曲及C_(6~7)椎体水平硬膜外积液、C_(7)硬膜缺损(例2)或侧脑室后角旁出血(例3);全脑血管造影无异常发现。临床诊断为幕下中枢神经系统表面铁沉积症(Ⅰ型2例、Ⅱ型1例),病因分别为神经鞘瘤、外伤致硬脊膜缺损或脑血管畸形。针对病因施行左侧C7硬膜缺损修补术(例2)或左侧枕叶血肿清除术(例3),术后病情明显好转,头痛症状消失。结论幕下中枢神经系统表面铁沉积症病因异质性较强,详尽的辅助检查对避免误诊或漏诊具有重要提示意义。Objective To report 3 cases of infratentorial superficial siderosis of central nervous system and analyze its etiology and clinical manifestations.Methods and Results The main clinical symptoms were recurrent headache or epilepsy(3 cases) with obvious hearing loss(2 cases);the appearance of cerebrospinal fluid of lumbar puncture was cloudy and bloody fluid,with increased red blood cell count and protein quantity(2 cases).MRI scan of head or cervical spine showed siderosis deposition on the surface of cerebellum hemisphere,pons and midbrain without enhancement,and occupying lesion at jugular foramen was seen at the same time(Case 1);tortuous vessels on the surface of cervical spinal cord,epidural effusion at the level of C_(6-7) vertebral body,C_(7) dural defect(Case 2) or paraventricular hemorrhage(Case 3).There were no abnormal findings in angiography of the whole brain.The clinical diagnosis was infratentorial superficial siderosis of central nervous system(type Ⅰ 2 cases,type Ⅱ 1 case).The causes were neurilemmoma,traumatic dural defect or cerebrovascular malformation.According to the cause of the disease,the left C_(7) dural defect repair(Case 2) or left occipital lobe hematoma removal(Case 3) were performed.The patients’ condition improved significantly and the headache disappeared.Conclusions The etiology of infratentorial superficial siderosis of central nervous system is heterogeneous.Detailed auxiliary examination is of great significance in avoiding misdiagnosis or missed diagnosis.

关 键 词：含铁血黄素沉着症 中枢神经系统 硬膜 脑脊髓液 磁共振成像 

分 类 号：R741[医药卫生—神经病学与精神病学]