儿童惊吓性癫的临床及脑电图特征

Clinical and Electroencephalogram Characteristics of Startle Epilepsy in Children

目的探讨儿童惊吓性癫的临床及脑电图特征。方法对2003年12月-2008年3月在北京大学第一医院儿科就诊的7例惊吓性癫患儿的病因、发作诱发因素、发作类型、脑电图特点、治疗及预后进行回顾性分析。结果 7例惊吓性癫患儿中男4例,女3例;起病年龄为5个月～7.5岁。有病因学异常6例,涉及4种不同的获得性病因;有影像学异常6例,以局部脑萎缩多见。7例均有惊吓性发作,同期有自发性发作,其中4例以自发性发作起病。发作的诱因5例为声音刺激,2例为碰触刺激。惊吓性发作的类型包括强直-不典型失神发作、肌阵挛发作、肌阵挛-强直发作、强直发作及局限性发作,与自发性发作类型可相同或不同。发作期脑电图常见弥散性电压衰减图形,并可见与发作类型相关的其他图形。病例对多种抗癫药的治疗均未见明显疗效。结论惊吓性癫常见为症状性的反射性癫,声音及碰触刺激为主要诱发因素,惊吓性发作包括全面性发作及局限性发作中的多种类型,发作期脑电图常见为弥散性电压衰减图形。本病对抗癫药治疗反应欠佳,总体预后差。

Objective To explore the clinical and electroencephalogram（EEG） characteristics of startle epilepsy in children.Methods The data of etiology,seizure stimuli and seizure types,EEG characteristics,treatment and prognosis of 7 patients were analyzed retrospectively,who were diagnosed as startle epilepsy between Dec.2003 to Mar.2008 in Department of Pediatrics,Peking University First Hospital.Results Of the 7 patients,3 cases were female,4 cases were male.The ages of onset were from 5 months to 7.5 years old.The abnormal etiology were found in 6 patients including 4 different acquired diseases.The abnormalities of imageology existed in 6 patients,in which focal cerebral atrophy was common.All the 7 patients encountered startle seizures with spontaneous seizures at the same time.The onset seizure types in 4 patients were spontaneous seizures.The provoking factors of seizures were sound（5 cases） and somatosensory（2 cases）.The startle seizures included tonic-atypical absence,myoclonic,myoclonic-tonic seizure,tonic seizure and partial seizure,which might be same as or different from the spontaneous seizures.The most common ictal EEG was diffuse electrodecremetal pattern.And other ictal EEG associated with the seizure types could also be seen.Multiple antiepileptic drugs were often unsatisfactory for all the patients.Conclusions Startle epilepsy is often symptomatic reflex epilepsy,and the provoking factors are mainly sound and somatosensory.The startle seizures included several types involving general and partial seizures.The ictal EEG often show diffuse electrodecremetal pattern.Startle epilepsy might have a poor response to antiepileptic drugs and a bad prognosis.