Lennox-Gastaut 综合征的临床表现和脑电图特点

目的 探讨Lennox-Gastaut综合征（LGS）的临床表现和脑电图特点。方法 62例LGS患儿首诊后,定期随诊临床发作和包括睡眠描记在内的脑电变化,根据临床和脑电图变化调整治疗。对首诊和随诊资料进行分析总结。结果 男37例,女25例,1-4岁为发病高峰（61%）,8岁以前发病为94%,43例（69%）为症状性癫痫,围产期损伤为突出的病因,19例（31%）为隐原性癫痫,患儿在病程中均有2种或2种以上发作形式,强直,不典型失神,点头和猝倒发作为特征性发作。都有不同程度的精神和智能发育迟缓,脑电图背景活动异常者为100%,均有特征性的双侧1-2.5次/s棘慢波综合,29例睡眠中出现双侧中短程爆发性10-14次/s快节律,主要根据发作类型。进行联合和个体化治疗,大部分病例能减少发作频率和减轻发作的严重程度。结论 LGS表现复杂,应重视其临床表现和脑电图特点,作出正确的诊断,及早进行积极而有效的治疗,最大程度上减少和减轻发作,以改善患儿的智能发育。

Characteristics of clinical manifestations and EEG of Lennox-Gastaut syndrome

OBJECTIVE: Lennox-Gastaut syndrome (LGS) is one of the most severe and refractory form of childhood epilepsy. The purpose of this study was to investigate the clinical and EEG characteristics of patients with LGS. METHODS: Sixty-two patients with LGS, including 37 males and 25 females, were followed-up regularly per three months or per six months, therapy was adjusted according to the changes in seizures and EEG, and the clinical data were analyzed in detail. RESULTS: The onset occurred between the age of 8 months and 12 years, with the peak at 1-4 years of age, accounting for 61%; a late onset which occurred after 8 years of age, was unusual. Furthermore, one patient who developed LGS at the age of 13 years and remained to have all the features of seizures and EEG at 35 years of age was identified as adult's LGS. Forty-three patients were classified as symptomatic, perinatal events were the predominant factors in this group. The others were cryptogenic. It was noted that 11 cases had a history of West syndrome. A transformation process from West syndrome to LGS was observed in another 7 cases. Every patient had two or more seizure types during the course of the disease; tonic seizure, atypical absence seizure, head drop or sudden falls were the characteristic types. The degree of mental deficit was variable from slight to profound deterioration, but mental and behavioral disturbances existed in every case as a rule. In all cases electroencephalogram (EEG) background was abnormal and consisted of diffuse slow spike-and-waves (1-1.5CPS), predominant in frontal and temporal regions. Twenty-four cases had the polyspike-wave. Bursts of fast rhythms (10-14CPS) were observed in 29 patients during sleep. The choice of antiepileptic drugs (AEDs) was based on the seizure types; routinely, 2 or more kinds of AEDs were used in combination, the classic drugs, valproate and clonazepam were firstly recommended; the other drugs, such as lamotrigine and topiramate that are used as add-on therapy were further consideration. Although the total effect was not satisfactory, the severity and frequency of seizures in almost all cases had lessened to some extent. CONCLUSION: LGS shows diverse manifestations; comprehensive diagnosis is crucial, active and efficacious treatment can improve the mental and behavioral development and prognosis as a whole.