摘要
目的分析结节性硬化症(TSC)患儿的临床、视频脑电图(VEEG)和影像学特征。方法对2000年6月-2010年6月在新疆维吾尔自治区人民医院儿科诊断为TSC患儿的临床资料,包括性别、年龄、民族构成、癫发作形式、皮肤损害情况和眼底情况等,以及头颅CT/MR I资料和VEEG。结果 41例TSC患儿中癫发作36例,癫的发生率为88.71%(36/41例),局限性发作为癫主要发作形式。VEEG异常者占77.78%(91/117例次),同一例患儿可见多种异常。TSC患儿头颅CT检查均有异常,均可见侧脑室外侧壁室管膜下结节状高密度影;23例TSC患儿头颅MR I异常,同一例患儿病变可以累及多个脑叶,并且病变范围较头颅CT更为广泛,头颅CT/MR I异常部位与VEEG的异常部位不完全相符。患儿眼底均未发现异常。结论 TSC患儿诊断主要依靠皮肤损害和头颅影像学检查,头颅CT/MR I检查对该病有诊断意义,但与VEEG的异常部位不完全相符,从经济学角度上认为行头颅CT检查更有价值。癫发作主要类型为局限性发作。
Objective To analyze the characteristics of clinic,video-electroencephalogram（VEEG） and imaging of children with tube-rous sclerosis complex（TSC）.Methods The clinics of 41 patients with TSC,including sex,age,ethnic group,seizure form,skin damage and eye end,VEEG and imaging data were reviewed.Results Seizure occurred in 36 cases out of the 41 patients（88.71%）,and abnormal VEEG appeared in 91 times out of all the 117 times VEEG（77.78%）.Abnormality was found in CT of all TSC patients,high-density images of subetendymal nodules were present in the lateral ventricle,and abnormality MRI was found in 23 patients,pathological changes in the same patient might involve more than one brain lobe,and the abnormality was found in more than one brain lobe in 1 patient.Abnormality ranged wider in head MRI than that in head CT,and the location of head CT/MRI was incompatible with that of the VEEG.Economically,applications of head CT were more valuable.Seizures were mainly of partial type.Conclusions TSC is mainly local and diagnosed on the basis of skin damage and skull imaging checks.Head CT/MRI had significant value in diagnosing this illness,but is not fully in line with the location of VEEG abnormality.Economically,applications of head CT are more valuable.Seizures are mainly of partial type.
出处
《实用儿科临床杂志》
CAS
CSCD
北大核心
2011年第7期524-526,共3页
Journal of Applied Clinical Pediatrics
关键词
结节性硬化症
临床
视频脑电图
计算机断层扫描术
磁共振成像
tuberous sclerosis complex
clinic
video-electroencephalogram
X-ray computed tomography
magnetic resonance imaging
