Exploring the Efficacy and Safety of Vagus Nerve Stimulation for the Treatment of Epilepsy in Patients With Sturge-Weber Syndrome: A Pilot Study

Zizhang Cheng; Weijin Sun; Kaiqiang Ma; Xiongfei Wang; Junhong Pan; Haowei Ma; Xintao Peng; Guoming Luan; Yuguang Guan

doi:10.1016/j.pediatrneurol.2024.12.016

Exploring the Efficacy and Safety of Vagus Nerve Stimulation for the Treatment of Epilepsy in Patients With Sturge-Weber Syndrome: A Pilot Study

Pediatr Neurol. 2025 Jan 3:164:35-40. doi: 10.1016/j.pediatrneurol.2024.12.016. Online ahead of print.

Authors

Zizhang Cheng¹, Weijin Sun¹, Kaiqiang Ma¹, Xiongfei Wang¹, Junhong Pan¹, Haowei Ma¹, Xintao Peng¹, Guoming Luan², Yuguang Guan³

Affiliations

¹ Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China.
² Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China; Beijing Key Laboratory of Epilepsy, Beijing, China; Center of Epilepsy, Beijing Institute of Brain Disorders, Collaborative Innovation Center for Brain Disorders, Capital Medical University, Beijing, China. Electronic address: [email protected].
³ Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing, China; Beijing Key Laboratory of Epilepsy, Beijing, China; Center of Epilepsy, Beijing Institute of Brain Disorders, Collaborative Innovation Center for Brain Disorders, Capital Medical University, Beijing, China. Electronic address: [email protected].

PMID: 39848141
DOI: 10.1016/j.pediatrneurol.2024.12.016

Abstract

Background: Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous disorder, often complicated by epilepsy. Approximately 50% of patients with SWS with epilepsy develop drug-resistant seizures, leaving limited treatment options. Vagus nerve stimulation (VNS) is a known therapy for refractory epilepsy, modulating neural activity to reduce seizures. This study examines the therapeutic outcomes, efficacy, and safety of VNS in five patients with SWS suffering from epilepsy.

Methods: A retrospective analysis of VNS treatment data from January 2021 to January 2022 in patients with SWS was conducted. Preoperative assessments included neuroimaging and video-electroencephalography monitoring. Cognitive function and quality of life were assessed using age-appropriate scales. VNS settings and seizure outcomes were recorded at different follow-up intervals. Seizure outcomes were classified using the modified Engel and McHugh classification. Cognitive function and quality of life were reassessed at two-year follow-up.

Results: Five patients, primarily pediatric, with seizure onset between age 0.5 and eight years, were included. After VNS therapy, all patients experienced a reduction in seizure frequency, with one patient becoming seizure free and three achieving a ≥50% reduction in seizures. Two children with cognitive impairments at baseline demonstrated cognitive improvements following treatment. All patients reported significant enhancements in quality of life. VNS was well tolerated, with no major adverse events reported.

Conclusions: VNS offers promising therapeutic benefits for epilepsy in patients with SWS, reducing seizure frequency, improving cognitive function in children, and enhancing quality of life with a favorable safety profile. Further research with larger sample sizes and control groups is warranted to validate efficacy and explore personalized treatment options.

Keywords: Drug-refractory epilepsy; Safety analysis; Sturge-Weber syndrome (SWS); Treatment efficacy; Vagus nerve stimulation (VNS).