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

Xie, Han; Ma, Jiayi; Ji, Taoyun; Liu, Qingzhu; Cai, Lixin; Wu, Ye

doi:10.3389/fneur.2022.951850

Cited by 7 publications

(5 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A total of 220 studies were identified through a comprehensive search of electronic databases and related bibliographies. The databases used for the search included Embase (150), Cochrane ( 7 ), PubMed ( 28 ) and CNKI ( 10 ). Based on the titles and abstracts, a total of 55 studies were deemed potentially eligible and selected for further evaluation.…”

Section: Resultsmentioning

confidence: 99%

“…It has also been approved as a long-term treatment for DRE in children ( 8 ). VNS, a non-pharmacological intervention that enhances seizure control, has also been employed in the treatment of patients with DS ( 9 , 10 ). Studies have shown that VNS appears to be beneficial for children with SCN1A gene abnormalities associated with refractory epilepsy ( 11 ).…”

Section: Introductionmentioning

confidence: 99%

“…Studies have shown that VNS appears to be beneficial for children with SCN1A gene abnormalities associated with refractory epilepsy ( 11 ). Although the VNS is considered a viable treatment option for patients with DS, tuberous sclerosis complex (TSC) or Rett syndrome, the effectiveness of VNS for DRE with other rare gene mutations remains uncertain ( 10 ). Relevant studies have demonstrated that VNS therapy is both safe and effective in focal, generalized and combined types of epilepsy.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Vagus nerve stimulation for the therapy of Dravet syndrome: a systematic review and meta-analysis

Chen,

Li,

Huang

2024

Front. Neurol.

View full text Add to dashboard Cite

ObjectiveDravet syndrome (DS) is a refractory developmental and epileptic encephalopathy characterized by seizures, developmental delay and cognitive impairment with a variety of comorbidities, including autism-like behavior, speech dysfunction, and ataxia. Vagus nerve stimulation (VNS) is one of the common therapies for DS. Here, we aim to perform a meta-analysis and systematic review of the efficacy of VNS in DS patients.MethodsWe systematically searched four databases (PubMed, Embase, Cochrane and CNKI) to identify potentially eligible studies from their inception to January 2024. These studies provided the effective rate of VNS in treating patients with DS. The proportions of DS patients achieving ≥50% reduction of seizure frequency were extracted from these studies. Meta-analyses were performed to respectively evaluate the efficacy of VNS for DS after 3, 6, 12, 18, 24 and 36 months.ResultsSixteen trials with a total of 173 patients were included. Meta-analyses showed that the pooled efficiency was 0.54 (95% CI 0.43–0.65) in the DS patients treated with VNS (p < 0.05). Meanwhile, the pooled efficiency respectively was 0.42 (95% CI 0.25–0.61), 0.54 (95% CI 0.39–0.69), 0.51 (95% CI 0.39–0.66), and 0.49 (95% CI 0.36–0.63) in the DS patients treated with VNS after 3, 6, 12 and 24 months (p < 0.05).ConclusionThis study suggests that VNS is effective in the treatment of DS. However, few studies have focused on VNS for DS, and there is a lack of high-quality evidence. Thus, high-quality randomized controlled trials are needed to confirm the efficacy of VNS in DS.

show abstract

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Vagus nerve stimulation for the therapy of Dravet syndrome: a systematic review and meta-analysis

Chen,

Li,

Huang

2024

Front. Neurol.

View full text Add to dashboard Cite

show abstract

“…However, some knowledge is provided by the use of vagus nerve stimulation in the treatment of refractory epilepsy. Patients with RTT who underwent VNS displayed some side effects such as decreased appetite and coughing/choking on food, similar to patients with different diagnoses but not an exacerbation of their baseline breathing irregularities or cardiac complications (Wilfong and Schultz, 2006;Xie et al, 2022). In addition, the non-invasive tVNS, not employed in patients with this syndrome yet, could minimize typical side effects of invasive stimulation and increase tolerability.…”

Section: Conclusion and Future Perspectivesmentioning

confidence: 88%

Cardiac autonomic control in Rett syndrome: Insights from heart rate variability analysis

et al. 2023

View full text Add to dashboard Cite

Rett syndrome (RTT) is a rare and severe neurological disorder mainly affecting females, usually linked to methyl-CpG-binding protein 2 (MECP2) gene mutations. Manifestations of RTT typically include loss of purposeful hand skills, gait and motor abnormalities, loss of spoken language, stereotypic hand movements, epilepsy, and autonomic dysfunction. Patients with RTT have a higher incidence of sudden death than the general population. Literature data indicate an uncoupling between measures of breathing and heart rate control that could offer insight into the mechanisms that lead to greater vulnerability to sudden death. Understanding the neural mechanisms of autonomic dysfunction and its correlation with sudden death is essential for patient care. Experimental evidence for increased sympathetic or reduced vagal modulation to the heart has spurred efforts to develop quantitative markers of cardiac autonomic profile. Heart rate variability (HRV) has emerged as a valuable non-invasive test to estimate the modulation of sympathetic and parasympathetic branches of the autonomic nervous system (ANS) to the heart. This review aims to provide an overview of the current knowledge on autonomic dysfunction and, in particular, to assess whether HRV parameters can help unravel patterns of cardiac autonomic dysregulation in patients with RTT. Literature data show reduced global HRV (total spectral power and R-R mean) and a shifted sympatho-vagal balance toward sympathetic predominance and vagal withdrawal in patients with RTT compared to controls. In addition, correlations between HRV and genotype and phenotype features or neurochemical changes were investigated. The data reported in this review suggest an important impairment in sympatho-vagal balance, supporting possible future research scenarios, targeting ANS.

show abstract

“…A previous study demonstrated that VNS therapy improved cognitive disorder, autism and quality of life (QOL) in low-IQ patients (18). Furthermore, VNS was effective in pediatric patients with drug-resistant epilepsy (DRE) of monogenic etiology (19,20). In CDKL5-DEE, seizure improvement was reported in more than two-thirds of patients post-VNS therapy, and additional benefits such as cognitive and behavioral improvements justify further use in other gene-induced DEEs (21).…”

Section: Introductionmentioning

confidence: 99%

Vagus nerve stimulation for treating developmental and epileptic encephalopathy in young children

Geng,

Hu,

Meng

et al. 2023

Front. Neurol.

View full text Add to dashboard Cite

ObjectiveTo 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.Patients and methodsWe 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.ResultsAmong 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).ConclusionsOur 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.

show abstract

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

Cited by 7 publications

References 25 publications

Vagus nerve stimulation for the therapy of Dravet syndrome: a systematic review and meta-analysis

Vagus nerve stimulation for the therapy of Dravet syndrome: a systematic review and meta-analysis

Cardiac autonomic control in Rett syndrome: Insights from heart rate variability analysis

Vagus nerve stimulation for treating developmental and epileptic encephalopathy in young children

Contact Info

Product

Resources

About