关键词: cognitive disorder developmental and epileptic encephalopathy drug-resistant epilepsy gene mutation vagus nerve stimulation

来  源:   DOI:10.3389/fneur.2023.1191831   PDF(Pubmed)

Abstract:
UNASSIGNED: To investigate the clinical variables that might predict the outcome of developmental and epileptic encephalopathy (DEE) after vagus nerve stimulation (VNS) therapy and identify the risk factors for poor long-term outcome.
UNASSIGNED: We retrospectively studied 32 consecutive children with drug-resistant DEE who had undergone VNS surgery from April 2019 to July 2021, which were not suitable for corpus callosotomy. In spite of combining valproic acid, levetiracetam, lamotrigine, topiramate, etc. (standard anti-seizure medicine available in China) it has not been possible to effectively reduce seizures in the population we investigate (Cannabidiol and brivaracetam were not available in China). A responder was defined as a frequency reduction decrease > 50%. Seizure freedom was defined as freedom from seizures for at least 6 months. Sex, electroencephalograph (EEG) group, neurodevelopment, time lag, gene mutation, magnetic resonance imaging (MRI), and epilepsy syndrome were analyzed with Fisher\'s exact test, The age at onset and age at VNS therapy were analyzed with Kruskal-Wallis test, statistical significance was defined as p < 0.05. And used the effect size to correction.
UNASSIGNED: Among the 32 patients, the median age at VNS implantation was 4.7 years (range: 1-12 years). At the most recent follow-up, five children (15.6%) were seizure-free and 22 (68.8%) were responders. Univariate analysis demonstrated that the responders were significantly associated with mild development delay/intellectual disability (p = 0.044; phi coefficient = 0.357) and a multifocal EEG pattern (p = 0.022; phi coefficient = -0.405). Kaplan-Meier survival analyses demonstrated that a multifocal EEG pattern (p = 0.049) and DEE without epileptic spasm (ES) (p = 0.012) were statistically significant (p = 0.030). Multivariate analysis demonstrated that DEE with ES had significant predictive value for poor long-term outcome (p = 0.014, hazard ratio = 5.433, confidence interval = 1.402-21.058).
UNASSIGNED: Our study suggested that VNS was a generally effective adjunct treatment for DEE. Although the predictive factors for VNS efficacy remain unclear, it should be emphasized that patients with ES are not suitable candidates for epilepsy surgery. Further investigations are needed to validate the present results.
摘要:
研究可能预测迷走神经刺激(VNS)治疗后发育性和癫痫性脑病(DEE)结果的临床变量,并确定长期预后不良的危险因素。
我们回顾性研究了从2019年4月至2021年7月接受VNS手术的连续32例耐药DEE儿童,这些儿童不适合进行骨体切开术。尽管结合了丙戊酸,左乙拉西坦,拉莫三嗪,托吡酯,等。(中国有标准的抗癫痫药物)在我们调查的人群中,无法有效减少癫痫发作(中国没有大麻二酚和布立西坦)。响应者被定义为频率减少减少>50%。癫痫发作自由定义为至少6个月的癫痫发作自由。性,脑电图(EEG)组,神经发育,时滞,基因突变,磁共振成像(MRI),癫痫综合征采用Fisher精确检验进行分析,使用Kruskal-Wallis检验分析VNS治疗的发病年龄和年龄,统计学显著性定义为p<0.05。并使用效果大小进行校正。
在32名患者中,VNS植入时的中位年龄为4.7岁(范围:1~12岁).在最近的随访中,5名儿童(15.6%)无癫痫发作,22名儿童(68.8%)为应答者.单变量分析表明,响应者与轻度发育迟缓/智力障碍(p=0.044;phi系数=0.357)和多灶性EEG模式(p=0.022;phi系数=-0.405)显着相关。Kaplan-Meier生存分析表明,多灶性EEG模式(p=0.049)和无癫痫性痉挛(ES)的DEE(p=0.012)具有统计学意义(p=0.030)。多变量分析表明,DEE与ES对不良长期结局具有显着的预测价值(p=0.014,风险比=5.433,置信区间=1.402-21.058)。
我们的研究表明,VNS通常是DEE的有效辅助治疗方法。尽管VNS疗效的预测因素尚不清楚,应该强调的是,ES患者不适合接受癫痫手术.需要进一步的调查来验证目前的结果。
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