CAJ | 학술논문

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。
목적：탐토중추신경계통표면철침적증적병인、병리궤제、림상화영상학특점급치료방법。방법보도1례경영상학학진적중추신경계통표면철침적증병례，병결합국내외문헌복습진행분석。결과환자남성，52세。만성기병，구유전형소뇌성공제실조、감음성이롱급척수병변삼련증，MRI T2시중추신경계통표면전형변연저신호영，진단위중추신경계통표면철침적증。결론중추신경계통표면철침적증주요고영상학진단，중국보도교소，목전상무유효치료방법。
Aim To investigate the etiology, pathomechanism, clinical features, neuro-imaging and treatment of superifcial 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 efifcacy and safety of the treatment remains to be investigated.