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Vagus nerve stimulation in children with drug-resistant epilepsy of monogenic etiology

Vagus nerve stimulation (VNS) is an effective treatment for drug-resistant epilepsy (DRE). The present study evaluated the efficacy of VNS in pediatric patients with DRE of monogenic etiology. A total of 20 patients who received VNS treatment at our center were followed up every 3 months through out...

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Autores principales: Xie, Han, Ma, Jiayi, Ji, Taoyun, Liu, Qingzhu, Cai, Lixin, Wu, Ye
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9475310/
https://www.ncbi.nlm.nih.gov/pubmed/36119689
http://dx.doi.org/10.3389/fneur.2022.951850
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author Xie, Han
Ma, Jiayi
Ji, Taoyun
Liu, Qingzhu
Cai, Lixin
Wu, Ye
author_facet Xie, Han
Ma, Jiayi
Ji, Taoyun
Liu, Qingzhu
Cai, Lixin
Wu, Ye
author_sort Xie, Han
collection PubMed
description Vagus nerve stimulation (VNS) is an effective treatment for drug-resistant epilepsy (DRE). The present study evaluated the efficacy of VNS in pediatric patients with DRE of monogenic etiology. A total of 20 patients who received VNS treatment at our center were followed up every 3 months through outpatient visits or a remote programming platform. The median follow-up time was 1.4 years (range: 1.0–2.9). The rate of response to VNS at 12 months of follow-up was 55.0% (11/20) and the seizure-free rate was 10.0% (2/20). We found that 75.0% (3/4) of patients with an SCN1A variant had a >50% reduction in seizure frequency. Patients with pathogenic mutations in the SLC35A2, CIC, DNM1, MBD5, TUBGCP6, EEF1A2, and CHD2 genes or duplication of X q28 (MECP2 gene) had a >50% reduction in seizure frequency. Compared with the preoperative electroencephalography (EEG), at 6, 12, 18, and 24 months after stimulator implantation, the percentage of the patients whose background frequency increased >1.5 Hz was respectively, 15.0% (3/20), 50.0% (10/20), 58.3% (7/12) and 62.5% (5/8); the percentage of the patients whose interictal EEG showed a >50% decrease in spike number was respectively 10% (2/20), 40.0% (8/20), 41.6% (5/12) and 50.0% (4/8). In the 9 patients with no response to VNS treatment, there was no difference in terms of spike number and background frequency between preoperative and postoperative EEG. Five of the 20 children (25.0%) reached new developmental milestones or acquired new skills after VNS compared to the preoperative evaluation. The efficacy of VNS in pediatric patients with DRE of monogenic etiology is consistent with that in the overall population of pediatric DRE patients. Patients with Dravet syndrome (DS), tuberous sclerosis complex (TSC), or Rett syndrome/MECP2 duplication syndrome may have a satisfactory response to VNS, but it is unclear whether patients with rare variants of epilepsy-related genes can benefit from the treatment.
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spelling pubmed-94753102022-09-16 Vagus nerve stimulation in children with drug-resistant epilepsy of monogenic etiology Xie, Han Ma, Jiayi Ji, Taoyun Liu, Qingzhu Cai, Lixin Wu, Ye Front Neurol Neurology Vagus nerve stimulation (VNS) is an effective treatment for drug-resistant epilepsy (DRE). The present study evaluated the efficacy of VNS in pediatric patients with DRE of monogenic etiology. A total of 20 patients who received VNS treatment at our center were followed up every 3 months through outpatient visits or a remote programming platform. The median follow-up time was 1.4 years (range: 1.0–2.9). The rate of response to VNS at 12 months of follow-up was 55.0% (11/20) and the seizure-free rate was 10.0% (2/20). We found that 75.0% (3/4) of patients with an SCN1A variant had a >50% reduction in seizure frequency. Patients with pathogenic mutations in the SLC35A2, CIC, DNM1, MBD5, TUBGCP6, EEF1A2, and CHD2 genes or duplication of X q28 (MECP2 gene) had a >50% reduction in seizure frequency. Compared with the preoperative electroencephalography (EEG), at 6, 12, 18, and 24 months after stimulator implantation, the percentage of the patients whose background frequency increased >1.5 Hz was respectively, 15.0% (3/20), 50.0% (10/20), 58.3% (7/12) and 62.5% (5/8); the percentage of the patients whose interictal EEG showed a >50% decrease in spike number was respectively 10% (2/20), 40.0% (8/20), 41.6% (5/12) and 50.0% (4/8). In the 9 patients with no response to VNS treatment, there was no difference in terms of spike number and background frequency between preoperative and postoperative EEG. Five of the 20 children (25.0%) reached new developmental milestones or acquired new skills after VNS compared to the preoperative evaluation. The efficacy of VNS in pediatric patients with DRE of monogenic etiology is consistent with that in the overall population of pediatric DRE patients. Patients with Dravet syndrome (DS), tuberous sclerosis complex (TSC), or Rett syndrome/MECP2 duplication syndrome may have a satisfactory response to VNS, but it is unclear whether patients with rare variants of epilepsy-related genes can benefit from the treatment. Frontiers Media S.A. 2022-09-01 /pmc/articles/PMC9475310/ /pubmed/36119689 http://dx.doi.org/10.3389/fneur.2022.951850 Text en Copyright © 2022 Xie, Ma, Ji, Liu, Cai and Wu. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neurology
Xie, Han
Ma, Jiayi
Ji, Taoyun
Liu, Qingzhu
Cai, Lixin
Wu, Ye
Vagus nerve stimulation in children with drug-resistant epilepsy of monogenic etiology
title Vagus nerve stimulation in children with drug-resistant epilepsy of monogenic etiology
title_full Vagus nerve stimulation in children with drug-resistant epilepsy of monogenic etiology
title_fullStr Vagus nerve stimulation in children with drug-resistant epilepsy of monogenic etiology
title_full_unstemmed Vagus nerve stimulation in children with drug-resistant epilepsy of monogenic etiology
title_short Vagus nerve stimulation in children with drug-resistant epilepsy of monogenic etiology
title_sort vagus nerve stimulation in children with drug-resistant epilepsy of monogenic etiology
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9475310/
https://www.ncbi.nlm.nih.gov/pubmed/36119689
http://dx.doi.org/10.3389/fneur.2022.951850
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