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癫痫儿童睡眠中出现的发作性对称性强直伸展姿势和发作后全面性 EEG 抑制。

Ictal Symmetric Tonic Extension Posturing and Postictal Generalized EEG Suppression Arising From Sleep in Children With Epilepsy.

机构信息

Division of Neurology, The Hospital for Sick Children, Department of Paediatrics, University of Toronto, Toronto, Ontario, M5G 1X8, Canada; Department of Pediatrics, Oita University Faculty and Medicine, Oita, 879-5593, Japan.

Division of Neurology, The Hospital for Sick Children, Department of Paediatrics, University of Toronto, Toronto, Ontario, M5G 1X8, Canada.

出版信息

Pediatr Neurol. 2017 Nov;76:54-59. doi: 10.1016/j.pediatrneurol.2017.06.018. Epub 2017 Jul 4.

Abstract

BACKGROUND

The identification of a biomarker for sudden unexpected death in epilepsy (SUDEP) has the potential to save lives. Generalized convulsive seizures and postictal generalized suppression on electroencephalography (EEG) most often precede sudden unexpected death in epilepsy (SUDEP) and are potential biomarkers. We identify the EEG and seizure characteristics associated with postictal generalized EEG suppression in children with epilepsy.

METHODS

Video EEGs were reviewed for seizure type, duration and semiology, and electrographic features. To identify predictors of postictal generalized EEG suppression, we identified 40 children with generalized convulsive seizures from a group of 399 patients who experienced an electroclinical seizure during video-EEG. Seventy-seven generalized convulsive seizures with and without postictal generalized EEG suppression were anayzed.

RESULTS

Age of seizure onset was older in 19 children with postictal generalized EEG suppression (mean 6.8 years old, 95% CI [4.7-8.9]) than in 21 without postictal generalized EEG suppression (3.0 years old, [1.1-4.1], P = 0.041). Postictal generalized EEG suppression occurred significantly more often from sleep than awake (point of estimate 16.67; 95% CI [0.97-32.36], P < 0.038). Shorter duration of the clonic phase (-0.76; [-1.338, -0.133], P = 0.018) was significantly associated with postictal generalized EEG suppression. Ictal symmetric tonic extension posturing significantly increased the odds of postictal generalized EEG suppression (42.94; [18.77, 67.12], P = 0.001). All 15 generalized convulsive seizures with a terminal burst-suppression pattern were followed by postictal generalized EEG suppression in contrast to 19 of 62 generalized convulsive seizures without burst-suppression (15.32, P < 0.001).

CONCLUSIONS

Ictal decerebrate-like symmetric tonic extension posturing with shorter clonic phase and a terminal burst-suppression pattern identify malignant generalized convulsive seizures, associated with postictal generalized EEG suppression and a potentially increased risk of sudden unexpected death in epilepsy.

摘要

背景

癫痫猝死(SUDEP)生物标志物的鉴定有可能挽救生命。全身性强直阵挛发作和脑电图(EEG)的发作后全面抑制通常先于癫痫猝死(SUDEP),是潜在的生物标志物。我们确定了与癫痫儿童发作后全面 EEG 抑制相关的 EEG 和发作特征。

方法

对视频 EEG 的发作类型、持续时间和症状学以及电描记图特征进行了回顾。为了确定发作后全面 EEG 抑制的预测因素,我们从经历视频-EEG 临床电发作的 399 名患者中确定了 40 名患有全面性强直阵挛发作的儿童。分析了 77 次伴有和不伴有发作后全面 EEG 抑制的全面强直阵挛发作。

结果

发作后全面 EEG 抑制的 19 名儿童的发病年龄(平均 6.8 岁,95%置信区间[4.7-8.9])大于 21 名无发作后全面 EEG 抑制的儿童(3.0 岁,[1.1-4.1],P=0.041)。发作后全面 EEG 抑制在睡眠中比清醒时更常见(估计点 16.67;95%置信区间[0.97-32.36],P<0.038)。阵挛相持续时间较短(-0.76;[-1.338,-0.133],P=0.018)与发作后全面 EEG 抑制显著相关。发作期对称强直伸展姿势显著增加发作后全面 EEG 抑制的几率(42.94;[18.77,67.12],P=0.001)。与无爆发抑制的 62 次全面强直阵挛发作中的 19 次相比,所有 15 次全面强直阵挛发作的终末爆发抑制模式均伴有发作后全面 EEG 抑制(15.32,P<0.001)。

结论

发作期去脑强直样对称强直伸展姿势,伴有较短的阵挛相和终末爆发抑制模式,可识别恶性全面强直阵挛发作,与发作后全面 EEG 抑制和癫痫猝死的潜在风险增加相关。

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