In this study, we present the electroclinical features and outcomes of 92 patients with epileptic spasms (ES) in clusters without modified or classical hypsarrhythmia that started in either in infancy or in childhood; we compared both groups in terms of electroclinical features, etiology, treatment, evolution, and outcome. Between June 2000 and July 2022, 92 patients met the electroclinical diagnostic criteria of ES in clusters without hypsarrhythmia. Patients with ES associated with other epileptic encephalopathies including West Syndrome, as well as those with the specific etiology of ES and developmental and epileptic encephalopathy associated with were excluded. The patients were divided into two groups based on the age at ES onset: those with ES onset before (Group 1) and those with ES onset after 2 years of age (Group 2). The features of ES and the type of associated seizures before and after ES onset, as well as the interictal and ictal EEG and electromyography findings were similar in both groups. The etiologies were mainly structural (40.2%), genetic (11.9%), and unknown (44.6%) in majority of the patients in both groups. Thirty-one patients were seizure-free, while in the remaining patients the seizures continued. Nine patients (9.8%) with unilateral structural lesions underwent surgery with good results. The neurological abnormalities and developmental findings prior to ES onset depended on the underlying etiology. Our series of patients may represent a well-defined epileptic syndrome or type of epilepsy with onset in infancy or childhood characterized by ES in clusters without hypsarrhythmia associated with focal and generalized seizures and EEG paroxysms without neurological deterioration.

中文翻译：

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婴儿期和儿童期不伴有高度心律失常的丛集性癫痫痉挛：单一年龄依赖性癫痫类型还是明确的癫痫综合征？


在这项研究中，我们介绍了 92 名成簇癫痫痉挛 (ES) 患者的电临床特征和结果，这些患者没有在婴儿期或儿童期开始出现改良型或经典型高度节律失常。我们比较了两组的电临床特征、病因、治疗、演变和结果。 2000年6月至2022年7月期间，有92名患者符合ES的电临床诊断标准，无高度节律失常。与其他癫痫性脑病（包括 West 综合征）相关的 ES 患者，以及具有 ES 特定病因以及与之相关的发育性和癫痫性脑病的患者被排除在外。根据 ES 发病年龄将患者分为两组：之前发生 ES 的患者（第 1 组）和 2 岁后发生 ES 的患者（第 2 组）。两组的 ES 特征和 ES 发作前后相关癫痫发作类型，以及发作间期和发作期脑电图和肌电图结果相似。两组大多数患者的病因主要是结构性（40.2%）、遗传性（11.9%）和未知（44.6%）。三十一名患者没有癫痫发作，而其余患者的癫痫发作仍在继续。 9例（9.8%）单侧结构性病变患者接受了手术，效果良好。 ES 发病前的神经系统异常和发育异常取决于潜在的病因。我们的系列患者可能代表一种明确的癫痫综合征或癫痫类型，在婴儿期或儿童期发病，其特征是成群的 ES，没有与局灶性和全身性癫痫发作相关的高度节律失常，以及脑电图阵发，但没有神经功能恶化。