25例结节性硬化患儿脑电图和影像学特征及临床疗效分析  

Characteristics of EEG,radiology and effect of clinical therapy in 25 children with tuberous sclerosis

作　　者：孙鲁妍[1] 崔华勤[1] 吕爱敏[1] 黄文波[1] 马风杰[1] 张贺[1] 

机构地区：[1]潍坊医学院附属医院电生理室,山东潍坊261031

出　　处：《临床神经电生理学杂志》2005年第4期202-205,共4页Journal of Clinical Electroneurophysiology

摘　　要：目的:分析结节性硬化(TS)患儿的脑电图(EEG)、影像学特征及临床治疗效果.方法:对25例TS患儿的EEG、CT/MRI资料及抗癫癎药物(AED)的治疗效果进行回顾性分析.结果:TS患儿癫癎发生率为100%,3岁之前发病者20例(80%),主要发作类型为部分性发作13例(52%).EEG异常21例(84%),其中19例(90%)有癎样放电.3例结节灶直径≥12 mm者其位置与EEG主要放电部位相符,MRI对皮质结节及白质病灶的显示非常敏感.短期AED治疗14例(56%)有效,15例随访1～11年,4例加重.结论:癫癎始发年龄小,EEG有局限性改变,对首次药物治疗反应不良且伴有智能障碍特征者,以及MRI上表现大量皮质结节的患儿,癫癎发作较难控制,易发展成为难治性癫癎.Objective.. To analyze the EEG, radiogical characteristics and the treatment of tuberous sclerosis（TS）. Methods：The data on EEG, CT/MRI and antiepilepsy drugs in 25 children with TS were analyzed retrospectively. Results：The incidence of epilepsy in all the cases was 100%. Epileptic seizures occurred before 3 years old in 20 cases （80 %）. Partial seizures belonged to the main seizure type in 13 cases（52%）, mental retardation was found in 17 cases. EEG was found abnormal in 21 children （84%）, and epileptiform discharge was showed in 19 cases （90%）. Local EEG changes were closely associated with the clinical localized sign. The cortical tubers （diameter ≥ 12mm） in CT tended to be correlated with the main discharges in EEG in 3 cases. Compared with CT, more tubers were found distinctly and MRI. Short-term AED therapy was effective in 14 cases （56%）. After 1 to 11 years＇ following-up, 4 ca- ses became even worse. Conclusions： In children with TS,if seizures begin at younger age, partial seizures have local epileptiform discharge in EEG, and unresponsive to the initial drug therapy with mental retardation and vast cortical tubers were shown in MRI, The cases are likely to transformed to intractable epilepsy.

关 键 词：结节性硬化 脑电图 磁共振成像 癫痫 临床 

分 类 号：R747.9[医药卫生—神经病学与精神病学] R741.044[医药卫生—临床医学]