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.
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