摘要
目的:总结我国中枢神经系统表面铁沉积症(superficial siderosis of central nervous system,SSCNS)患者的临床、影像学特征和治疗情况。方法:回顾性收集于2015—2019年就诊于山东大学齐鲁医院的4例经磁共振成像(MRI)和磁敏感加权成像(SWI)确诊的SSCNS患者,总结其临床资料,分析其病因及影像学特点等。结果:4例患者均为男性,平均年龄60.5岁。临床症状主要包括头痛、听力下降和小脑性共济失调。颅脑MRI及SWI所显示的含铁血黄素沉积部位主要包括小脑、脑干、颞叶、额叶、脊髓,显示为线条状短T 2信号,SWI低信号。4例患者均发现可能的出血部位,其中1例有颅脑外伤出血史,1例有脊髓髓外硬膜下海绵状血管瘤,1例脑转移瘤,1例颅内动脉瘤。对患者进行为期6个月至4年的随访,仅1例行手术治疗者头痛症状好转,余3例对症治疗者症状均进展。结论:SSCNS主要表现为听力下降、进行性小脑共济失调和脊髓病变,诊断主要依靠影像学检查,磁共振T 2WI及SWI神经系统表面线性低信号是诊断的主要依据。该病目前以手术治疗出血原发病及铁螯合剂等药物治疗为主。
Objective To explore the etiology,clinical features and treatment of superficial siderosis of central nervous system(SSCNS)in China.Methods The clinical data of four patients with SSCNS diagnosed by magnetic resonance imaging(MRI)and susceptibility weighted imaging(SWI)from Qilu Hospital of Shandong University during 2015—2019 were retrospectively reviewed.The etiology and clinical features of the four cases were summarized and analyzed.Results All the four patients were male,with an average age of 60.5 years.Clinical symptoms included headache,hearing loss,and cerebellar ataxia.Brain MRI and SWI showed that hemosiderin mainly deposited(short linear T2 signal,low SWI signal)on the surface of cerebellum,brainstem,temporal lobe,frontal lobe,and spinal cord.Potential bleeding sites were found in all four patients,including brain traumatic history,spinal intradural extramedullary cavernous hemangioma,brain metastasis and intracranial aneurysm.Patients were followed up for six months to four years.Headache symptoms improved in only one patient who received surgical treatment,while symptoms of the other three patients progressed.Conclusions SSCNS is mainly characterized by hearing loss,progressive cerebellar ataxia and myelopathy.The diagnosis of this disease mainly depends on imaging examination.The linear low signal on the surface of T2WI is the main basis for the diagnosis of SSCNS.Surgical treatment of bleeding sites and iron chelator are the main treatments of the disease.
作者
左瑶
贾国勇
孟桂月
刘颖
王翠兰
Zuo Yao;Jia Guoyong;Meng Guiyue;Liu Ying;Wang Cuilan(Department of Neurology,Qilu Hospital of Shandong University,Jinan 250012,China)
出处
《中华神经科杂志》
CAS
CSCD
北大核心
2020年第4期291-297,共7页
Chinese Journal of Neurology
