Abstract：
OBJECTIVE: 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.
METHODS: 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 CDKL5 were excluded.
RESULTS: 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.
CONCLUSIONS: 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.
METHODS: 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 CDKL5 were excluded.
RESULTS: 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.
CONCLUSIONS: 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.
摘要：
目的：在本研究中，我们介绍了92例癫痫性痉挛（ES）患者的临床特征和结果，这些患者在婴儿期或儿童期均无改良或经典心律失常；我们比较了两组的临床特征，病因学,治疗,进化,和结果。
方法：在2000年6月至2022年7月之间，92例患者符合ES的电临床诊断标准，无心律失常。与其他癫痫性脑病（包括West综合征）相关的ES患者，与CDKL5相关的发育性和癫痫性脑病以及具有ES特定病因的患者均被排除.
结果：根据ES发病年龄将患者分为两组：ES发病前（第1组）和ES发病后（第2组）。ES发病前后的特征及相关癫痫发作类型,两组的发作间期和发作期脑电图和肌电图检查结果相似。病因主要为结构性（40.2%），遗传（11.9%），和未知（44.6％），在大多数的两组患者。31名患者没有癫痫发作，而在其余患者中，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发现取决于潜在的病因。
结论：我们的一系列患者可能代表了一种明确的癫痫综合征或癫痫类型，在婴儿期或儿童期发作，以ES为特征，成群，没有与局灶性和全身性癫痫发作相关的心律失常，也没有脑电图发作，没有神经系统恶化。
方法：在2000年6月至2022年7月之间，92例患者符合ES的电临床诊断标准，无心律失常。与其他癫痫性脑病（包括West综合征）相关的ES患者，与CDKL5相关的发育性和癫痫性脑病以及具有ES特定病因的患者均被排除.
结果：根据ES发病年龄将患者分为两组：ES发病前（第1组）和ES发病后（第2组）。ES发病前后的特征及相关癫痫发作类型,两组的发作间期和发作期脑电图和肌电图检查结果相似。病因主要为结构性（40.2%），遗传（11.9%），和未知（44.6％），在大多数的两组患者。31名患者没有癫痫发作，而在其余患者中，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发现取决于潜在的病因。
结论：我们的一系列患者可能代表了一种明确的癫痫综合征或癫痫类型，在婴儿期或儿童期发作，以ES为特征，成群，没有与局灶性和全身性癫痫发作相关的心律失常，也没有脑电图发作，没有神经系统恶化。
