Abstract：
The incidence of epileptic spasms (ES) that begin after the first year of life is much lower than that before 1 year of age. The aim of this study was to identify clinical and electroencephalography (EEG) characteristics, etiologies, treatments, and prognoses in pediatric patients with ES onset after 1 year of age. Forty-one children were retrospectively identified in Children\'s Hospital of Chongqing Medical University between January 1, 2020 and December 1, 2021. ES onset after 1 year of age have diverse presentations. Although most occur in clusters, are symmetrical and flexional, and occur frequently during awakening, some are characterized as isolated and asymmetrical, have a tonic component, and can also occur during sleep. The hypsarrhythmia variants and focal or multifocal discharges occur alternately in the interictal period, and the focal spikes and slow waves predominated in the unilateral temporal or frontotemporal areas. These patients had diverse etiologies, including structural (51.2 % of patients) and genetic (22.0 %) ones, and 11 patients (26.8 %) had an unknown etiology. No patients in our study had an infectious or immune-mediated etiology. Forty-eight percent of patients responded to hydrocortisone and/or adrenocorticotropic hormone. The efficacy of antiepileptic drug therapy was lower in patients who did not receive concurrent steroid therapy. However, ES onset after 1 year of age caused by a tumor, brain malformation, or other focal lesions, may be cured by focal cortical resection despite a lack of clearly localized EEG surface anomalies. Delays in motor, language, and cognitive development, or behavioral problems were observed in all but three patients.
摘要：
在生命的第一年之后开始的癫痫性痉挛（ES）的发病率远低于1岁之前的发病率。这项研究的目的是确定临床和脑电图（EEG）特征，病因，治疗,1岁后发生ES的儿科患者的预后。2020年1月1日至2021年12月1日，重庆医科大学附属儿童医院对41名儿童进行了回顾性分析。1岁后的ES发病有不同的表现。虽然大多数发生在集群中，对称和屈曲，在觉醒过程中经常发生，有些是孤立和不对称的，有一个补品成分，也可能发生在睡眠期间。心律失常变异型和局灶性或多灶性放电在发作间期交替发生，局灶性尖峰和慢波在单侧颞区或额颞区占主导地位。这些患者有不同的病因，包括结构性（51.2%的患者）和遗传性（22.0%），11例(26.8%)病因不明。在我们的研究中，没有患者具有传染性或免疫介导的病因。48％的患者对氢化可的松和/或促肾上腺皮质激素有反应。在未接受同步类固醇治疗的患者中，抗癫痫药物治疗的疗效较低。然而,1岁后由肿瘤引起的ES发病，大脑畸形,或其他局灶性病变，尽管缺乏明确的局部EEG表面异常，但仍可通过局灶性皮质切除术治愈。电机延迟,语言,和认知发展，除三名患者外，所有患者均出现行为问题。
