Lennox-Gastaut综合征合并孤独症与智力发育障碍的临床特征及危险因素分析  

Clinical features and risk predictors of autism and intellectual disability in patients with Lennox-Gastaut syndrome

作　　者：何娜[1] 黎冰梅[1] 汪洁[1] 刘晓蓉[1] 李斌[1] 卞文君 区锶文 高志炜 廖卫平[1] 汤斌[1] He Na;Li Bingmei;Wang Jie;Liu Xiaorong;Li Bin;Bian Wenjun;Ou Siwen;Gao Zhiwei;Liao Weiping;Tang Bin(Institute of Neuroscience,Second Affiliated Hospital of Guangzhou Medical University,Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and Ministry of Education of China,Collaborative Innovation Center for Neurogenetics and Channelopathies,Guangzhou 510260,China)

机构地区：[1]广州医科大学附属第二医院神经内科和神经科学研究所,神经致病基因与离子通道病省部共建教育部重点实验室,广州510260

出　　处：《中华神经医学杂志》2019年第4期381-386,共6页Chinese Journal of Neuromedicine

基　　金：国家自然科学基金(81571274);广东省教育厅项目(2013LYM_0071).

摘　　要：目的探讨Lennox-Gastaut综合征(LGS)患者中孤独症、智力发育障碍的共患发生率、临床特点及临床影响因素。方法收集广州医科大学附属第二医院癫痫中心自2012年6月至2018年6月确诊为LGS的患者64例,采用儿童孤独症家长评定量表(ABC)和孤独症医生评定量表(CARS)对患儿进行孤独症评估;采用《中国儿童韦氏智力量表》(C-WISC)和《Gesell儿童发育诊断量表》对患儿进行智力发育评估,分析不同临床因素对LGS并发孤独症及智力发育障碍的影响。结果64例LGS患者中仅3例(4.7%)患者合并有孤独症,其平均ABC和CARS评分分别为80分和40分。不同年龄分组中,起病年龄<1岁患者ABC和CARS评分最高,分别为(40.9±26.7)分和(26.0±8.9)分,与其他年龄组患者相比,差异有统计学意义(P<0.05)。治疗药物数量分组中,服用≥3种抗癫痫药物(AEDs)患者ABC和CARS评分最高,分别为(27.8±22.8)分和(22.2±8.7)分;与服用1~2种AEDs患者相比,差异有统计学意义(P<0.05)。此外,不同癫痫发作频率、具有/不具有明确病因的LGS患者,ABC和CARS评分差异也具有统计学意义(P<0.05)。64例患者中50例(78.1%)患者呈现不同程度的智力障碍,其中重度智力障碍为主要类型,占31.3%。轻度、中度以及极重度智力障碍患者人数分别为12(18.8%)、7(10.9%)和11(17.2%)。与不伴智力发育障碍患者比较,伴有智力障碍患者起病年龄更低、脑电图特征表现为慢背景活动比例更高、具有症状性病因的比例更高,差异有统计学意义(P<0.05)。结论癫痫起病年龄早,癫痫发作频繁,服用≥3种AEDs且具有明确病因的LGS患者合并孤独症的几率更高。起病年龄是LGS患者合并智力障碍的独立危险因素。Objective To investigate the co-occurrence incidence,clinical features and risk predictors of autism and intellectual disability in patients with Lennox-Gastaut syndrome(LGS).Methods Sixty-four patients with LGS were recruited in our Epilepsy Center from June 2012 to June 2018.Autism Behavior Checklist(ABC)and Childhood Autism Rating Scale(CARS)were performed to evaluate autism,while Chinese Wechsler Intelligence Scale for Children(C-WISC)and Gesell Developmental Scale were applied to estimate intelligence.The influences of different clinical factors in autism and intellectual disability were analyzed in patients with LGS.Results Among 64 patients with LGS,only three(4.7%)were diagnosed as having autism,and their average ABC and CARS scores were 80.0 and 40.0,respectively.The average ABC and CARS scores were 40.9±26.7 and 26.0±8.9 in the patients with onset age<one year,which were significantly higher than those in other two groups,respectively(P<0.05).The average ABC and CARS scores in the patients accepted antiepileptic drugs(AEDs)≥3 were 27.8±22.8 and 22.2±8.7,which were significantly higher than those in the patients accepted one or two kinds of AEDs(P<0.05).In addition,the ABC and CARS scores showed significant differences in the groups with different seizure frequency and in the groups with or without symptomatic etiologies(P<0.05).Fifty patients(78.1%)presented different levels of intellectual disability;severe intellectual disability was the leading type,which accounted for 31.3%(20/64);12(18.8%),7(10.9%),and 11(17.2%)patients were with mild,moderate or profound intellectual disability,respectively.As compared with patients without intellectual disability,patients with intellectual disability had younger onset age,higher proportion of slow background activity on EEG and higher proportion of symptomatic etiologies,with significant differences(P<0.05).Conclusion Patients are in higher risk of autism when they have earlier epilepsy onset age,higher frequency of epilepsy seizure attack,administration of

关 键 词：LENNOX-GASTAUT综合征 孤独症 智力发育障碍 危险因素 

分 类 号：R749.94[医药卫生—神经病学与精神病学] R742.1[医药卫生—临床医学]