儿童结节性硬化症合并疒间性发作的临床特征及治疗(20例病例分析)  被引量:1

Clinical characteristics and treatment of twenty cases diagnosed with Tuberous Sclerosis with seizures as the first symptom.

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作  者:赵骋[1] 王华[1] 

机构地区:[1]中国医科大学附属盛京医院小儿神经内科,110004

出  处:《辽宁医学杂志》2016年第4期60-63,共4页Medical Journal of Liaoning

摘  要:目的探讨儿童结节性硬化(Tuberous sclerosis)合并疒间性发作的临床特征及治疗。方法对2011年1月至2015年4月中国医科大学附属盛京医院小儿神经内科20例以疒间性发作为首发症状住院治疗,且临床诊断为儿童结节性硬化患儿的临床表现、影像学特点、脑电图检查等临床资料进行回顾性分析。结果患者年龄2月龄至7岁(平均29.4月龄),均以疒间性发作为住院首发症状(100%),发病≤1周就诊者9例(45%),发病≤6月、>1周就诊者8例(40%),发病>6月就诊者3例(15%)。有皮肤损害者15例(75%)。头CT检查者14例(70),头MRI检查者18例(90%),同时行头CT及头MRI检查者12例。全部病例均有影像学改变。完善视频脑电图者12例(60%)、动态脑电图6例(30%),普通脑电图8例(40%),其中发现异常者16例(75%),确诊癫疒间16例。心脏彩超异常3例;肾脏彩超异常2例;结论 3岁以下结节性硬化伴抽搐发作患儿多以癫疒间发作、皮肤损害为主要临床特征。颅脑CT、MRI和脑电图检查对该疾病的诊断有重要价值。皮肤损害者出现癫疒间发作后应尽早就诊,早期完善头部CT或MRI检查明确诊断,并且早期完善视频或动态脑电图明确有无合并癫疒间,对患儿病情发展及治疗有重要意义。Objective To explore the clinical characteristics and treatment of twenty cases diagnosed with Tuberous Sclerosis with seizures as the first symptom. Methods The clinical features, including clinical manifestations, imaging data (head MRI/CT) ,EEG/AEEG/VEEG examination data,in 20 patients with seizures as the first symptom of hospitalization who diagnosed with Tuberous Sclerosis were retrospectively analyzed. Results The age of onset was 2 months to 7 years old ( the average age of onset is 29.4 months old). each case was hospitalized with seizures as the first symptom. The disease course was less than one week is 9 eases(45% ). The disease course of 8 cases was between one week to six months (40%) ;Three cases were over six months. There are 15 cases with skin damage(75% ). 14 cases did head CT examina- tion,18 cases did head MRI examination(90% ),12 cases did both. Each case had imaging changes. 8 cases did EEG examination, 12 cases did VEEG examination,and 6 cases did AEEG examination. Abnormal EEG was 16 cases and all these cases were diagnosed with Epilepsy. 3 cases showed abnormalities in Doppler echocardiography. 2 cases showed abnormalities in Renal Uhrasonography. Conclusion Children under 3 years of age who diagnosed with Tuberous Sclerosis with epilepsy were often recognized of seizures and skin lesions as the main clinical features . Head CT, MRI and EEG data are sig- nificant valuable for the diagnosis. Patients who had skin lesions and convulsion should visit hospital as soon as possible. Head CT and MRI can make definite diagnosis of Tuberous Sclerosis and EEG examination can diagnose epilepsy during the early period, which play a key role in improving the prognosis of disease.

关 键 词:结节性硬化 癫疒间 婴儿痉挛 儿童 

分 类 号:R725.9[医药卫生—儿科]

 

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