中国实用儿科杂志2011，Vol.26Issue(11)：832-835,4.
肌阵挛-站立不能性癫痫临床及脑电图特征分析
Clinical and electroencephalographic features of myoclonic-astatic epilepsy
林希 1包新华 2刘晓燕 2杨志仙 2常杏芝 2符娜 2熊晖 2王爽 2秦炯2
作者信息
- 1. 福建医科大学附属第一医院儿科,福州 350004
- 2. 北京大学第一医院儿科,北京 100034
- 折叠
摘要
Abstract
Objective To investigate the clinical and electroencephalographic characteristics and treatment of myoclonic-astatic epilepsy (MAE). Methods Twelve MAE cases who were diagnosed during 2006-2010 in Peking University First Hospital were enrolled in this study. The clinical and electroencephalographic (EEG) characteristics, treatment and prognoses of MAE were analyzed. Results Among 12 cases, eleven were male and only one was female. Five of them had family history of febrile convulsion or epilepsy. Age at presentation ranged from 15 to 45 months (average 32.3 months).All the patients showed normal development before the onset of epilepsy and no abnormality was found in brain MRI.A11 the MAE patients had multiple generalized seizure types including tonic-clonic, myoclonic-astatic, myoclonic, atonic, atypical absence and tonic seizures. Myoclonic-astatic and atypical absence seizures were present in all cases.Six patients had a history of non-convulsive status epilepticus.The interictal EEG showed diffused slow wave background in 10 cases,and generalized spike and wave discharges (no focal discharges) in all patients.Valproate was the common drug choice which was often combined with other antiepileptic drugs.ACTH may be effective.The time of follow-up ranged from 1 month to 4 years and 7 months.Seizures were controlled from 1 months to 3 years in 7 cases and not completely controlled in 5 cases.Seven patients showed normal intelligence and five showed mental retardation after onset. Conclusion MAE is one of idiopathic generalized epileptic syndrome in childhood characterized by myoclonic-astatic seizure.NCSE often occurred in MAE.Clinical observation, ictal video-EEG and electromyography (EMG) are essential to the diagnosis of MAE.Valproate alone or combined with other antiepileptic drugs such as lamotrigine could have a favorable effect on MAE.The prognosis of MAE is various.关键词
肌阵挛-站立不能性癫痫/诊断/治疗/预后Key words
myoclonic-astatic epilepsy/ diagnosis/ therapy/ prognosis分类
医药卫生引用本文复制引用
林希,包新华,刘晓燕,杨志仙,常杏芝,符娜,熊晖,王爽,秦炯..肌阵挛-站立不能性癫痫临床及脑电图特征分析[J].中国实用儿科杂志,2011,26(11):832-835,4.
