摘要
目的 探讨结节性硬化症所致癫 痫患儿的CT诊断及临床特点。方法 回顾 性分析和总结30例TSC合并癫痫患儿的临 床资料及CT表现。结果 CT检查均可见侧 脑室外侧室壁管膜下有结节状高密度影, 大小不一,密度均匀,其中22例脑室内 有结节状高密度影,4例大脑半球皮质存 在结节状高密度影,2例白质内有多发结 节状高密度影。30例患儿,17例<1岁,6 例1-3岁,5例4-6岁,2例>6岁;20例单 一发作,7例为2种同时发作,3例为3种同 时发作;25例伴随皮肤损害,18例出现智 力障碍,27例EEG异常。结论 CT结合结节 性硬化症所致癫痫患儿的临床表现、EEG 等,可作为临床诊断结节性硬化症所致癫 痫的重要影像学方式。
Objective To explore CT diagnosis and clinical characteristics of children with epilepsy induced by tuberous sclerosis. Methods The clinical data and CT findings of 30 children with TSC complicated with epilepsy were retrospectively analyzed and summarized. Results CT examination showed that there was nodular high density shadow under periosteum of external ventricle wall of the lateral ventricle, variable in size with homogeneous density. There were 22 cases with nodular high density shadow in cerebral ventricle, 4 cases with nodular high density shadow in cerebral cortex of hemicerebrum and 2 cases with nodular high density shadow in white matter. Among the 30 cases of patients, 17 cases were younger than 1 years old, 6 cases 1-3 years old, 5 cases 4-6 years old, 2 case over 6 years old; 20 cases were single seizure, 7 cases 2 kinds of seizures at the same time and 3 cases 3 kinds of seizures at the same time; There were 25 cases complicated with skin lesions, 18 cases with mental retardation and 27 cases with abnormal EEG. Conclusion CT combined with clinical characteristics and EEG of children with epilepsy induced by tuberous sclerosis can be used as an important imaging method for the diagnosis of epilepsy induced by nodular sclerosis
【关键词】结节性硬化症;癫痫;临床特 点;头颅CT;影像学表现
【中图分类号】R445.3;R596.1
【文献标识码】A
【DOI】 10.3969/j.issn.1672- 5131.2016.01.007
前言
结节性硬化症(TSC)为常染体显性遗传性疾病,发病率为3.3/10万 [1]。儿童发病期常表现为癫痫发作、皮肤受、智力低下等,部分患者 可能累及重要脏器官、视网膜,临床表现多样。多项研究结果显示, 大部分TSC患者均存在癫痫发作时,常表现为抽搐。有文献指出,儿童 癫痫发病时间、频率与智力受损呈正相关关系,即癫痫发病年龄越早 或发病频次越多,智力损害程度越严重。然而,目前国内关于TSC合并 癫痫的临床研究存在不足,可能影响疾病治疗。笔者回顾性分析医院 明确诊断的30例TCS致癫痫患儿的临床资料及CT表现,为临床诊断和治 疗TSC合并癫痫提供资料,现报道如下。
中国CT和MRI杂志
第14卷, 第 1 期
2016年01月
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