中枢神经系统表面铁沉积症的临床分析  

Superficial siderosis of the central nervous system:A literature analysis and case report

作　　者：周若琳 孙莉[1] 王梓铖 齐一鸣 张海宁[1] ZHOU Ruolin;SUN Li;WANG Zicheng(Diagnosis and Treatment Center of Cognitive Disorders,Department of Neurology,the First Hospital of Jilin University,Changchun 130021,China)

机构地区：[1]吉林大学白求恩第一医院神经内科认知障碍疾病诊疗中心,吉林长春130021

出　　处：《中风与神经疾病杂志》2023年第12期1089-1095,共7页Journal of Apoplexy and Nervous Diseases

基　　金：吉林省医疗卫生人才专项(JLSWSRCZX2021-077)。

摘　　要：目的探讨中枢神经系统表面铁沉积症(SSCNS)的病因、临床表现、影像学特点及治疗等。方法报道1例确诊的SSCNS患者,并检索中国知网、万方数据库,对确诊的61例SSCNS患者,进行流行病学、病因、临床表现、实验室检查的分类统计。结果文献报道的61例SSCNS患者中,临床症状以共济失调最为常见(49例,80%),其次为听力下降(43例,70%)、锥体束征(30例,49%)。其中25例(39%)找到了可能的病因。结论SSCNS病程可达数年甚至数十年,临床症状主要表现为听力障碍、共济失调和锥体束征。磁共振成像(MRI)是最具价值的诊断依据,磁敏感加权成像(SWI)技术对于微出血诊断的敏感性更高。Objective To investigate the etiology,clinical manifestations,imaging features,and treatment of superficial siderosis of the central nervous system(SSCNS).Methods A patient confirmed with SSCNS was reported.Sixtyone patients confirmed with SSCNS were found through a search of China National Knowledge Infrastructure and Wanfang Data and were analyzed in categories for the epidemiology,etiology,clinical manifestations,and laboratory test results.Results Among the 61 patients with SSCNS reported in the literature,ataxia was the most common clinical symptom(49 cases,80%),followed by hearing loss(43 cases,70%)and pyramidal signs(30 cases,49%).A probable cause was found in 25(39%)of these cases.Conclusion SSCNS can last for several years or even decades,with hearing loss,ataxia,and pyramidal signs as its main clinical symptoms.Magnetic resonance imaging is the most valuable basis for diagnosis,and susceptibility-weighted imaging is more sensitive in the diagnosis of microhemorrhage.

关 键 词：中枢神经系统 含铁血黄素沉积 共济失调 

分 类 号：R743[医药卫生—神经病学与精神病学] R744[医药卫生—临床医学]