CNS表面含铁血黄素沉积症的临床及CSF细胞学特点(附2例报告)  

Clinical and CSF cytological features of superficial siderosis of CNS( report of 2 cases)

作　　者：李淑敏[1] 孙巧凤[1] 王翠[1] 蔺建文[1] 赵红玲[1] 王虹[1] 刘赞华[1] 王苏平[1] 

机构地区：[1]大连医科大学附属大连市中心医院神经内科,116033

出　　处：《临床神经病学杂志》2015年第6期463-465,共3页Journal of Clinical Neurology

摘　　要：目的探讨CNS表面含铁血黄素沉积症(SSCNS)的临床和CSF细胞学特点。方法回顾性分析2例SSCNS患者的临床资料。结果本组2例患者均为慢性病程,进行性加重,均有听力下降,双侧病理征(+),例2有左侧肢体共济失调,Romberg征(+)。增强头颅MRI示T2WI/Flair脑表面均见弥漫,线状低信号影覆盖伴线性强化。CSF细胞学检查均有异常,均发现红细胞吞噬细胞及含铁血黄素吞噬细胞。结论SSCNS临床表现为感音性耳聋、锥体束征及小脑性共济失调等。头颅MRI在T2WI上有特征性改变。CSF细胞学检查可见红细胞吞噬细胞及含铁血黄素吞噬细胞。Objective To explore the clinical and CSF cytological features of superficial siderosis of CNS（ SSCNS）. Methods The clinical data of 2 patients with SSCNS were retrospectively reviewed. Results Two cases showed chronic onset,progressive aggravation,and had hearing loss and pyramidal tract sign,case 2 had left limb ataxia and Romberg sign（ ＋）. Both T_2 WI / Flair of enhanced cranial MRI showed brain surface was covered by linear low signal widely,with linearly enhancement. They both had abnormalities in CSF cytology with erythrocyte phagocytic cells and hemosiderin phagocytic cells. Conclusions SSCNS is characterized by hearing loss,pyramidal tract sign and cerebellar ataxia,and so on. T_2 WI of cranial MRI has characteristic change. Cerebrospinal fluid cytology with erythrocyte phagocytic cells and hemosiderin phagocytic cells could also be found.

关 键 词：CNS表面含铁血黄素沉积症 CSF细胞学 临床特点 

分 类 号：R741[医药卫生—神经病学与精神病学]