癫痫脑病伴慢波睡眠期持续棘慢波发放三例  

The study of three patients with epileptic encephalopathy with continuous spike and waves during slow wave sleep

作　　者：李婷 贾宇 张礼萍[1] 魏荣[1] 王玉平[2,3] 戚小红[1] Li Ting;Jia Yu;Zhang Liping;Wei Rong;Wang Yuping;Qi Xiaohong(Department of Pediatrics,Xuanwu Hospital,Capital Medical University,Beijing 100053,China)

机构地区：[1]首都医科大学宣武医院儿科,北京100053 [2]首都医科大学宣武医院神经内科,北京100053 [3]脑功能疾病调控治疗北京市重点实验室,首都医科大学脑重大疾病研究院癫痫所

出　　处：《脑与神经疾病杂志》2022年第11期661-665,共5页Journal of Brain and Nervous Diseases

基　　金：北京市医院管理中心儿科学科协同发展中心儿科专项一般项目(XTYB201810)。

摘　　要：目的分析3例癫痫脑病伴慢波睡眠期持续棘慢波(CSWS)发放患者的临床症状,脑电图(EEG),影像学表现和治疗后转归。方法纳入2013年9月至2016年9月于首都医科大学宣武医院儿科收治3例CSWS患者,收集并分析患者的临床资料,总结该疾病特点。结果3例患儿,均为男童,平均就诊年龄为6岁(5.2~6.9岁),发病年龄为4岁。患儿均有癫痫发作,发作类型包括不典型失神,肌阵挛发作,失张力发作,局灶性发作伴意识障碍等多种形式。所有患儿发病后均出现神经心理和/或运动功能发育倒退。EEG提示具备特征性慢波睡眠期持续性棘慢波发放,放电指数>85%。头颅MRI检查中2例(例1、例3)患儿有结构性异常。所有患者进行智力筛查,韦氏智力量表提示智力低下,分数平均为62(60~65)分。结合患者的临床表现及辅助检查,均满足CSWS诊断条件。诊断明确后均予以口服抗癫痫药物及激素治疗,随访平均4.5(3.5~5)年,患儿的临床发作次数明显减少,2例(例2、例3)患者临床无发作。神经认知状况和运动功能均较前有所改善。复查时所有患儿的EEG检测放电指数较前减少,1例(例3)患儿癫痫样放电消失。结论CSWS是一种具有特征性EEG的癫痫综合征,学龄期男童较常见,早期诊断及治疗可有效的减低发作频率,改善患儿神经心理和运动功能。Objective To summarize and analyze the clinical symptoms,abnormal electroencephalogram(EEG),magnetic resonance imaging(MRI)features and treatment prognosis of patients with epileptic encephalopathy with continuous spike and waves during slow wave sleep(CSWS).Methods The clinical information of 3 patients with CSWS admitted to the Department of Pediatrics,Xuanwu Hospital,Capital Medical University from September 2013 to September 2016 were collected and analyzed.Results Three patients were all boys,with an median age of 6 years.All3 children had seizures,including atypical absence,myoclonic seizures,atonic seizures,focal seizures with disturbance of consciousness,etc.Neuropsychological and/or motor functional regression occurred in all children after onset.The EEG indicated characteristic electrical status epileptics during slow wave sleep(ESES)and the discharge index was more than 85%.MRI showed structural abnormalities in 2 cases.All patients were screened for mental retardation on the Wechsler Intelligence Scale,with an average score of 60.Combined with the clinical manifestations and auxiliary examination of patients,all patients met the diagnosis of CSWS.After diagnosis,all patients were given oral antiepileptic drugs and glucocorticoid therapy.Followed up for 4.5(3.5-5)years,the clinical seizures were significantly reduced,and 2 patients had seizures free.Neurocognitive status and motor function were improved.The electroencephalogram discharge index of all the children decreased,and no abnormal epileptic discharge was found in 1 child.Conclusion CSWS is a kind of epilepsy syndrome with characteristic EEG,which is more common in school-age boys.Early diagnosis and treatment can effectively reduce the frequency of seizures and improve the neuropsychological and motor functions of children.

关 键 词：癫痫脑病伴慢波睡眠期持续棘慢波发放 智力低下 慢波睡眠期持续性棘慢波 放电指数 糖皮质激素 

分 类 号：R742.1[医药卫生—神经病学与精神病学]