小儿结节性硬化的临床特征与脑部CT评价  

The clinical features and CT evaluation of cerebral tuberous sclerosis in children

作　　者：李国栋[1] 吕京光[2] 董春华[3] 赵建设[3] 张凤霞[2] 

机构地区：[1]山东省苍山县人民医院CT室,山东苍山277700 [2]山东省医学影像学研究所,山东济南250021 [3]山东大学齐鲁儿童医院,山东济南250022

出　　处：《医学影像学杂志》2008年第7期717-719,共3页Journal of Medical Imaging

摘　　要：目的:探讨结节性硬化的病因、病理、临床表现、CT诊断与鉴别诊断。方法:本文对32例小儿经临床证实为结节性硬化患者的临床与脑部CT资料进行了回顾性分析。结果:32例小儿患者临床表现为癫痫发作25例,智力障碍17例,皮脂腺瘤13例,同时有以上三项表现者7例,有两项者18例,有一项或其它表现者7例。32例CT均表现为突入脑室内的室管膜下结节,钙化性结节18例,无钙化结节9例,两种结节同时存在5例。脑皮层及皮层下多发钙化结节9例;灰质异位与白质脱髓鞘改变3例;脑发育不良7例。结论:结节性硬化具有特征性的临床与CT表现,CT扫描直接全面地显示该病的脑部特征性表现,对本病的诊断与鉴别诊断具有很大的价值。Objective：To investigate the etiology,pathology,clinical features,CT diagnosis,and differential diagnosis of tuberous sclerosis.Methods：CT manifestations and clinical data of 32 cases in children with clinically diagnosed as cerebral tuberous sclerosis were analysed retrospectively.Results：Of the clinical manifestations of 32 cases in children,seizures were found in 25 cases,mental deficiency in 17 cases,sebaceous adenoma in 13 cases,and three manifestations above in 7 cases,two above in 18 cases,only one or other manifestations above in 7 cases.CT findings of 32 cases were subependymal nodules in all,which extruded lateral ventricles,with calcification in nodules in 18 cases,without calcification in 9 cases,both with and without calcification in 5 cases.There were cortical and subcortical tubercle with calcification in 9 cases,and heterotopic gray matter and myelinolysis in 3 cases,subependymal giant-celled astrocytoma located near the left interventricular foramina in 1 case,encephalodysplasiain 7 cases.Conclusion：There are characteristic clinical manifestations and CT findings in tuberous sclerosis.CT scaning directly shows all appearances of this disease and is of great value for diagnosing and differential diagnosing tuberous sclerosis.

关 键 词：小儿 结节性硬化 钙化 体层摄影术 X线计算机 

分 类 号：R593.9[医药卫生—内科学] R814.42[医药卫生—临床医学]