Encephalopathy with status epilepticus during sleep or continuous spikes and waves during slow sleep syndrome: A multicenter, long-term follow-up study of 117 patients

Encephalopathy with status epilepticus during sleep or continuous spikes and waves during slow sleep syndrome: A multicenter, long-term follow-up study of 117 patients
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DOI:
10.1016/j.eplepsyres.2013.02.010
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发表时间:
2013-07-01
期刊:
影响因子:
2.2
通讯作者:
Cersosimo, Ricardo
Cersosimo, Ricardo
中科院分区:
医学4区
文献类型:
--
作者:
Horacio Caraballo, Roberto;Veggiotti, Pierangelo;Cersosimo, Ricardo

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目的:回顾性分析117例长期随访的癫痫持续状态脑病(ESES)或持续性棘波缓慢睡眠(CSWSS)综合征患者的电临床特征、病因、治疗和预后。方法:分析1990年至2012年随访的117例ESES/CSWSS综合征患者的病历。纳入标准为:(1)局灶性癫痫发作或明显全身性癫痫发作且脑电图局灶性癫痫样放电; (2) 进一步发生非典型失神、肌阵挛、失张力和/或全身性癫痫发作; (3) 认知障碍和/或行为障碍; (4)超过85%的非快速眼动睡眠中的慢速睡眠期间出现持续的棘波放电。还分析了慢速睡眠中出现低于 85% 的棘波放电的患者。 主要发现:“在由 79 名儿童组成的症状性/结构性和非特发性组中,从 ESES/CSWSS 发病起的平均随访时间为 13 年(范围,2-22 年),在由 38 名儿童组成的特发性组中,平均随访时间为 10.5 年(范围,2.-21 年)。”症状性/结构性组和非特发性组与特发性组在不同阶段(ESES/CSWSS之前、期间和之后)的临床表现和阵发性脑电图异常(局灶性、多灶性或全身性)的定位以及ESES/CSWSS期间棘波指数的百分比的比较没有统计学意义。意义:ESES/CSWSS综合征是一种癫痫性脑病,在儿童中具有相似的电临床表现具有 >85% 尖峰波指数和具有
Purpose: To retrospectively analyze the electroclinical features, etiology, treatment and prognosis of 117 patients with encephalopathy with status epilepticus during steep (ESES) or continuous spike and waves slow sleep (CSWSS) syndrome with a long-term follow-up.Methods: 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.Key findings: '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.Significance: ESES/CSWSS syndrome is an epileptic encephalopathy with similar electroclinical findings in children with a >85% spike-wave index and those with a