中枢神经系统表面铁质沉积症的病因和临床表现分析

目的探讨中枢神经系统表面铁质沉积症(SSCNS)的病因及临床特点,以提高对该病的认识。方法收集临床确诊的53例SSCNS,根据铁沉积部位分为两种亚型[皮质型(cSS)及幕下型(iSS)],分析这两种亚型的病因、临床表现。结果53例患者中,cSS32例(60.4%),iSS21例(39.6%)。有明确病因47例(88.7%),无明显病因6例(11.3%)。cSS的主要病因包括:脑小血管病22例(68.8%),其中脑淀粉样血管病(CAA)14例(43.8%);脑皮层梗死5例(15.6%);慢性硬膜下血肿2例(6.3%);外伤性脑实质出血、蛛网膜下腔出血(SAH)2例(6.3%)。iSS的主要病因包括脑小血管病4例(19.0%);血管畸形3例(14.3%);颅脑术后3例(14.3%);白血病2例(9.5%);另有无明显原因6例(28.6%)。iSS的主要临床表现有:锥体束征、小脑共济失调、进行性双耳神经性听力下降;cSS患者表现为反复头晕。结论临床上多种引起SAH的病因均可导致SSCNS,但仍有一部分患者原因不明;cSS与iSS在病因及临床表现上均有所不同,脑小血管病尤其CAA是导致cSS的主要原因;iSS有特异性临床表现,但发生率不高,cSS缺乏特异性临床表现,SSCNS确诊有赖于MR检查。

Objective To explore the etiology and clinical features of superficial siderosis of the central nervous system(SSCNS)to improve the understanding of the disease.Methods Altogether 53 SSCNS patients were collected and divided into two subtypes according to the location of iron deposition:cortical type(cSS)and infratentorial type(iSS).The etiology and clinical manifestations of these two subtypes were analyzed retrospectively.Results Of the 53 patients,32(60.4%)were cSS and 21(39.6%)were iSS.Forty-seven cases(88.7%)with clear etiology,and 6 cases(11.3%)with no obvious etiology.The main causes of cSS included cerebral microvascular disease in 22 cases(68.8%),cerebral amyloid angiopathy(CAA)in 14 cases(43.8%);cerebral infarction in 5 cases(15.6%);chronic subdural hematoma in 2 cases(6.3%);traumatic parenchymal hemorrhage and subarachnoid hemorrhage(SAH)were found in 2 cases(6.3%).The main etiology of iSS included cerebral microvascular disease in 4 cases(19.0%);vascular malformation in 3 cases(14.3%);craniocerebral operation in 3 cases(14.3%);leukemia in 2 cases(9.5%),and no obvious cause in 6 cases(28.6%).The main clinical manifestations of iSS were pyramidal tract sign,cerebellar ataxia,and progressive binaural hearing loss.The patients with cSS presented with recurrent dizziness.Conclusion A variety of clinical causes of SAH can lead to SSCNS,but there are still some patients with unknown causes.There are differences in etiology and clinical manifestations between cSS and iSS.Cerebrovascular disease,especially CAA,is the main cause of cSS.ISS has specific clinical manifestations,but the incidence is not high.CSS lacks specific clinical manifestations,and the diagnosis of SSCNS depends on MR examination.