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伦诺克斯-加斯托综合征的治疗策略:多模式治疗方法的结果

Treatment strategies for Lennox-Gastaut syndrome: outcomes of multimodal treatment approaches.

作者信息

Na Ji-Hoon, Jung Da Eun, Kang Hee Jung, Kang Hoon-Chul, Kim Heung Dong

机构信息

Division of Pediatric Neurology, Department of Pediatrics, Epilepsy Research Institute, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.

Department of Pediatrics, Ajou Hospital, Ajou University School of Medicine, Suwon, Republic of Korea.

出版信息

Ther Adv Neurol Disord. 2022 Aug 3;15:17562864221108012. doi: 10.1177/17562864221108012. eCollection 2022.

DOI:10.1177/17562864221108012
PMID:35958038
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9358332/
Abstract

BACKGROUND

Multimodal treatment approaches are often considered for patients with Lennox-Gastaut syndrome (LGS). Creating an algorithm that can guide healthcare providers in selecting treatment options for patients with LGS remains a challenge. Herein, we assessed the long-term seizure-free and neurodevelopmental outcomes of stepwise multimodal treatment in patients with LGS.

OBJECTIVE

Herein, we assess the long-term seizure-free and neurodevelopmental outcomes of stepwise multimodal treatment in patients with LGS.

METHODS

We retrospectively examined the data of 371 patients with LGS who underwent stepwise multimodal treatment, including antiseizure medication (ASM) therapy, dietary therapy (DT), resective epilepsy surgery (R-ES), and palliative epilepsy surgery (P-ES). The seizure-free outcome was considered to be the effect of the final treatment according to the treatment algorithm, and the percentage of patients who remained seizure-free in each treatment group was calculated. ASM treatment, DT, R-ES, and P-ES were applied to 371 (100%), 201 (54.2%), 112 (30.2%), and 115 (31.0%) patients with LGS, respectively. We evaluated the stepwise multimodal treatment outcomes in these patients.

RESULTS

One hundred sixty-eight patients (45.3%) remained seizure-free for at least 1 year (seizure-free-for-1-year group), 61 of whom (16.5%) remained seizure-free for more than 5 years (remained-seizure-free group). Among the patients treated with ASM therapy, DT, R-ES, and P-ES, 41 (11.1%), 53 (14.3%), 56 (15.1%), and 29 (7.8%), respectively, remained seizure-free for 1 year. In addition, 15 (4.1%), 15 (4.1%), 19 (5.1%), and 12 (3.2%) patients in the ASM, DT, R-ES, and P-ES treatment groups, respectively, remained seizure-free for more than 5 years. Both the seizure-free-for-1-year and remained-seizure-free groups showed significant improvement in electroencephalography findings and neurodevelopmental status following treatment.

CONCLUSION

This study provides an update on the long-term seizure outcomes and neurodevelopmental improvements in a large cohort of patients with LGS following comprehensive multimodal treatment. We emphasize that the active combination of multiple ASMs, DT, and surgical treatment could provide long-term seizure-free outcomes and significant neurological benefits to patients with LGS.

摘要

背景

对于 Lennox-Gastaut 综合征(LGS)患者,通常会考虑采用多模式治疗方法。创建一种能够指导医疗保健提供者为 LGS 患者选择治疗方案的算法仍然是一项挑战。在此,我们评估了 LGS 患者逐步多模式治疗的长期无癫痫发作和神经发育结局。

目的

在此,我们评估 LGS 患者逐步多模式治疗的长期无癫痫发作和神经发育结局。

方法

我们回顾性分析了 371 例接受逐步多模式治疗的 LGS 患者的数据,包括抗癫痫药物(ASM)治疗、饮食疗法(DT)、切除性癫痫手术(R-ES)和姑息性癫痫手术(P-ES)。无癫痫发作结局被视为根据治疗算法的最终治疗效果,并计算每个治疗组中无癫痫发作患者的百分比。ASM 治疗、DT、R-ES 和 P-ES 分别应用于 371 例(100%)、201 例(54.2%)、112 例(30.2%)和 115 例(31.0%)LGS 患者。我们评估了这些患者的逐步多模式治疗结局。

结果

168 例患者(45.3%)至少 1 年无癫痫发作(1 年无癫痫发作组),其中 61 例(16.5%)无癫痫发作超过 5 年(持续无癫痫发作组)。在接受 ASM 治疗、DT、R-ES 和 P-ES 治疗的患者中,分别有 41 例(11.1%)、53 例(14.3%)、56 例(15.1%)和 29 例(7.8%)1 年无癫痫发作。此外,ASM、DT、R-ES 和 P-ES 治疗组中分别有 15 例(4.1%)、15 例(4.1%)、19 例(5.1%)和 12 例(3.2%)患者无癫痫发作超过 5 年。1 年无癫痫发作组和持续无癫痫发作组在治疗后的脑电图检查结果和神经发育状况均有显著改善。

结论

本研究提供了一大群 LGS 患者在综合多模式治疗后的长期癫痫发作结局和神经发育改善情况的最新信息。我们强调,多种 ASM、DT 和手术治疗的积极联合可为 LGS 患者提供长期无癫痫发作的结局和显著的神经学益处。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ced9/9358332/a003581a5d17/10.1177_17562864221108012-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ced9/9358332/21836f8becef/10.1177_17562864221108012-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ced9/9358332/0403d8f33022/10.1177_17562864221108012-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ced9/9358332/a003581a5d17/10.1177_17562864221108012-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ced9/9358332/21836f8becef/10.1177_17562864221108012-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ced9/9358332/0403d8f33022/10.1177_17562864221108012-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ced9/9358332/a003581a5d17/10.1177_17562864221108012-fig3.jpg

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