儿童伴不典型失神发作的临床及脑电图特征

Clinical and EEG characterstics of children with atypical absence seizures

目的探讨儿童不典型失神的电-临床特征、诊断及治疗。方法收集2011年1月—2016年12月山东大学齐鲁医院儿科神经门诊及住院的43例伴有不典型失神发作患儿的临床及EEG资料进行分析,随访治疗效果及预后。结果患儿男24例,女19例,平均5.43岁;起病年龄1岁8个月~10岁3个月不等。43例患儿均行头颅核磁共振(MRI)检查,18例未见明确异常,25例MRI存在异常:大脑皮质发育异常、脑萎缩先13例,天性胼胝体发育不良2例,双侧壳核后部异常信号2例,脑软化灶4例,脑室扩大2例,脑积水先2例。所有患儿不典型失神发作几乎都在清醒期,并均在院接受过1次以上脑电图(EEG)监测,所有患儿EEG均有较慢的背景活动,不典型失神发作期均见广泛性1.5~2.8 Hz棘慢波、慢棘慢复合波发放。随访所有患儿,6例发作完全控制,19例家长自述无发作,18例治疗效果差。40例存在不同程度的神经心理损伤及运动倒退,其中13例发病时已有发育迟缓;27例发病前认知近乎正常,患病2年后认知障碍的比例升至75%~95%。另3例损伤不明确。结论伴有不典型失神发作的大多数儿童智力损害及认知障碍的发生率较高,发病年龄较小,并且呈渐进性,在发育过程中有非特异性的脑损伤。抗癫痫药物可减少临床发作,但对神经心理损伤及运动倒退改善不明显。

Objective To investigate the clinical and EEG characteristics, therapeutic response and prognosis in children with atypical absence seizures. Methods The clinical and EEG data of 43 children with atypical absence seizures in Qilu Hospital, Shandong University during January 2011 to December 2016 were analyzed, and therapeutic response and prognosis were followed up. Results Childre were 24 male and 19 female with the mean age of 5.43 y. The onset ages were from 1 years and 8 months to 10 years and 3 months. All of the 43 patients had MRI examines, and 18 children were normol. MRI abnormalities appeared in 25 children, including cerebral cortical dysplasia and cerebral atrophy（13 cases）, congenital corpus callosum hypoplasia（2 cases）, and abnormal signal in bilateral posterior putamen（2 cases）, encephalomalacia focus（4 cases）, ventricle expention（2 cases）, hydrocephalus（2 cases）. All the children underwent EEG more than once. All children had atypical absence seizures during daytime. Children had slowly backgrounds in retesting EEG, and spine and slow waves of 1.5 Hz to 2.8 Hz could be seen in all the atypical absence seizures. All children were followed up, and except 6 children with complete control, 19 children＇ parents reported seizure-free, 18 children have poor effect. Forty Children had various degrees of psychological abnormalities and motor regression. Among them 13 cases had psychological abnormalities and motor regression before disease; cognitive ability of 27 cases were normal before onset age, however, about 75% to 95% of the children became abnormal 2 years after atypical absence seizures. The rest 3 cases had no obviously impairment. Conclusions Most of the atypical absence seizures children had small onset age and high incidence in mental damage and cognitive impairment. The course of typical absence seizures aggravate gradually, and often develops to nonspecific brain damage in this process. Antiepileptic drugs can reduce the frequency of the seizure in part of the patients, but had no effect on psychological and motor regression.