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目的:分析结节性硬化症(tuberous sclerosis complex,TSC)继发癫痫的儿童临床表现、影像学及脑电图(electroencephalogram,EEG)等特点,提高对该病的诊治水平。方法 :收集2008年5月~2014年2月在南京医科大学附属南京儿童医院诊断为TSC患儿的临床资料,包括性别、年龄、家族史、癫痫发作形式,皮肤损害,头颅CT、MRI、EEG和疗效等。结果:48例患儿中因癫痫发作首次就诊38例(79.17%),部分性发作多见,皮肤损害以色素脱失斑最多见,头颅CT、MRI检查阳性率100%,3例有家族史,治疗相对困难。结论:该病的诊断主要依靠癫痫发作、皮肤损害和影像学检查,癫痫为TSC患儿首次就诊的主要原因,发作控制困难,应长期随访。
Objective: To analyze the clinical manifestations, imaging and electroencephalogram (EEG) in children with secondary epilepsy of tuberous sclerosis complex (TSC) and to improve the diagnosis and treatment of the disease. Methods: The clinical data of TSC patients diagnosed as TSC in Nanjing Children’s Hospital affiliated to Nanjing Medical University from May 2008 to February 2014 were collected, including gender, age, family history, seizures, skin lesions, head CT, MRI, EEG And efficacy and so on. Results: The first visit of 38 cases (79.17%) occurred in 48 cases of children with seizures. The partial seizures were more common. The most common skin lesion was pigmented depigmentation lesion. The positive rate of CT and MRI was 100%, and 3 cases had family history , Treatment is relatively difficult. Conclusion: The diagnosis of the disease depends mainly on seizures, skin lesions and imaging examination. Epilepsy is the primary reason for the first visit of TSC. The onset of seizures is difficult to control and should be followed up for a long time.