中枢神经系统表面铁沉积症1例报道及文献复习.pdfVIP

中国临床神经科学 2015 年第 23 卷第 2 期 ·156· Chin J Clin Neurosci 2015, 23 (2), 156~160 论 著 1 贺 旻，费国强，毛悦时 (复旦大学附属中山医院神经内科 200032) 关键词　中枢神经系统表面铁沉积症；小脑性共济失调；感音性耳聋 摘要 目的 探讨中枢神经系统表面铁沉积症的病因、病理机制、临床和影像学特点及治疗方法。方法 报道1 例经影像 学确诊的中枢神经系统表面铁沉积症病例，并结合国内外文献复习进行分析。结果 患者男性，52 岁。慢性起病，具有 典型小脑性共济失调、感音性耳聋及脊髓病变三联症，MRI T2 示中枢神经系统表面典型边缘低信号影，诊断为中枢神经 系统表面铁沉积症。结论 中枢神经系统表面铁沉积症主要靠影像学诊断，中国报道较少，目前尚无有效治疗方法。 A Case of Superficial Siderosis of the Central Nervous System and Literature Review HE Min, FEI Guo-qiang, MAO Yue-shi Department of Neurology, Zhongshan Hospital, Fudan University, Shanghai 200032, China KEY WORDS superficial siderosis of central nervous system; cerebella ataxia; sensorineural hearing loss ABSTRACT Aim To investigate the etiology, pathomechanism, clinical features, neuro-imaging and treatment of superficial siderosis of central nervous system (SSCNS). Methods An MRI diagnosed case of SSCNS was presented and the literature was reviewed. Results The case was a 52-year old man diagnosed of SSCNS presented with the classical clinical symptoms including cerebellar ataxia, sensorineural hearing loss and myelopathy, whose T2-weighed MRI showed characteristic marginal hypo-intensity around the central nervous system (CNS). Conclusion SSCNS is mainly diagnosed by MRI, few cases were reported in China. The efficacy and safety of the treatment remains to be investigated. 中枢神经系统表面铁沉积症(superfical siderosis of 制、影像学表现和治疗方法。 central nervous system ，SSCNS)是一种少见病。其发病 [1] 病例资料 率约0.7% 。因慢性、反复的蛛网膜下腔出血致红细胞 崩解后的代谢产物含铁血黄素沉积于软脑膜及其毗邻的 患者住院号：809 ×××，男性，52岁。因“渐进 脑实质