Servicio de Neurología, Hospital de Pediatría Prof. DR. Juan P. Garrahan, Buenos Aires, Argentina.
Epilepsy Res. 2013 Jul;105(1-2):164-73. doi: 10.1016/j.eplepsyres.2013.02.010. Epub 2013 Mar 16.
To retrospectively analyze the electroclinical features, etiology, treatment and prognosis of 117 patients with encephalopathy with status epilepticus during sleep (ESES) or continuous spike and waves slow sleep (CSWSS) syndrome with a long-term follow-up.
Charts of 117 patients with ESES/CSWSS syndrome followed between 1990 and 2012 were analyzed. Inclusion criteria were: (1) focal seizures or apparently generalized seizures and focal EEG epileptiform discharges; (2) further occurrence of atypical absences, and myoclonic, atonic, and/or generalized seizures; (3) cognitive impairment and/or behavior disturbances; (4) continuous spike-and-wave discharges during slow sleep in more than 85% of non-REM sleep. Patients with spike-and-wave discharges in less than 85% of slow sleep were also analyzed.
'Mean follow-up from onset of ESES/CSWSS was 13 years (range, 2-22 years) in the symptomatic/structural and non-idiopathic group consisting of 79 children and 10.5 years (range, 2-21 years) in the idiopathic group consisting of 38 children. The comparison of clinical findings and localization of paroxysmal EEG abnormalities (focal, multifocal, or generalized) at the different stages (before, during, and after ESES/CSWSS) and the percentage of spike-wave index during ESES/CSWSS between the symptomatic/structural and non-idiopathic and the idiopathic group was not statistically significant.
ESES/CSWSS syndrome is an epileptic encephalopathy with similar electroclinical findings in children with a >85% spike-wave index and those with a <85% spike-wave index. In this series of patients, the most commonly used treatments were clobazam, ethosuximide, sulthiame, alone or in combination. In refractory cases, high-dose steroids were administered. Among the AED responders, the idiopathic cases returned to normality and the structural cases returned to baseline cognitive development.
通过长期随访，回顾性分析 117 例睡眠中癫痫持续状态（ESES）或连续尖慢波睡眠（CSWSS）综合征患者的临床电生理特征、病因、治疗和预后。
分析了 1990 年至 2012 年间随访的 117 例 ESES/CSWSS 综合征患者的图表。纳入标准为：（1）局灶性发作或明显全身性发作，伴有局灶性 EEG 痫性放电；（2）进一步出现非典型失神发作，以及肌阵挛、失张力和/或全身性发作；（3）认知障碍和/或行为障碍；（4）非快速眼动睡眠期连续尖慢波放电超过 85%。也对慢波睡眠中尖慢波放电少于 85%的患者进行了分析。
“有症状/结构性和非特发性组包括 79 例儿童，从 ESES/CSWSS 发病到开始的平均随访时间为 13 年（范围为 2-22 年），特发性组包括 38 例儿童，平均随访时间为 10.5 年（范围为 2-21 年）。在不同阶段（ESES/CSWSS 之前、期间和之后）比较临床发现和阵发性 EEG 异常（局灶性、多灶性或全身性）的定位以及 ESES/CSWSS 期间尖波指数在有症状/结构性和非特发性与特发性组之间无统计学意义。”
ESES/CSWSS 综合征是一种癫痫性脑病，具有 >85%尖波指数和 <85%尖波指数的儿童具有相似的临床电生理表现。在本系列患者中，最常用的治疗方法是氯巴占、乙琥胺、硫喷妥钠，单独或联合使用。在难治性病例中，给予大剂量类固醇。在 AED 反应者中，特发性病例恢复正常，结构性病例恢复到基线认知发育水平。
