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Published on: January 7, 2019
Vagal nerve stimulation in tuberous sclerosis complex patients
D Parain1, M J Penniello, P Berquen
1Department of Neurophysiology, Centre Hospitalier Universitaire, Rouen, France.
Insights
Vagal nerve stimulation (VNS) effectively reduced seizures in children with tuberous sclerosis complex (TSC) and refractory epilepsy. While VNS is a well-tolerated adjunctive therapy, epilepsy surgery offered superior seizure control outcomes.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neuroscience
Background:
- Tuberous sclerosis complex (TSC) is a genetic disorder often associated with medically refractory epilepsy.
- Vagal nerve stimulation (VNS) is an established treatment for refractory epilepsy, but its efficacy in TSC requires further investigation.
Purpose of the Study:
- To evaluate the effectiveness and safety of intermittent vagal nerve stimulation (VNS) in pediatric patients with tuberous sclerosis complex (TSC) and drug-resistant epilepsy.
- To compare VNS outcomes in TSC patients with literature data, control VNS patients, and epilepsy surgery cohorts.
Main Methods:
- Retrospective, open-label, multicenter study involving ten pediatric patients with TSC and refractory epilepsy treated with VNS for at least six months.
- Comparison of seizure frequency reduction and adverse events with historical controls, registry data, and published surgical series.
Main Results:
- Nine out of ten TSC patients experienced at least a 50% reduction in seizure frequency with VNS.
- Half of the patients achieved a 90% or greater seizure frequency reduction; no adverse events were reported.
- VNS demonstrated improved seizure control compared to published VNS data and registry controls, but epilepsy surgery showed better outcomes.
Conclusions:
- Intermittent vagal nerve stimulation (VNS) is an effective and safe adjunctive therapy for managing refractory epilepsy in children with tuberous sclerosis complex (TSC).
- Epilepsy surgery presents a more promising option for achieving significant seizure control in this patient population.
Abstract:
This is an open-label, retrospective, multicenter study to determine the outcome of intermittent stimulation of the left vagal nerve in children with tuberous sclerosis complex and medically refractory epilepsy. The records of all children treated with vagal nerve stimulation were reviewed in five pediatric epilepsy centers to locate those with tuberous sclerosis complex who had been treated with vagal nerve stimulation for at least 6 months. These patients were compared with (1) a series of patients obtained from the literature, (2) 10 similar control patients with epilepsy obtained from a registry of patients receiving vagal nerve stimulation, and (3) four published series of tuberous sclerosis complex patients whose epilepsy was surgically managed. Ten tuberous sclerosis complex patients with medically refractory epilepsy treated with vagal nerve stimulation were found. Nine experienced at least a 50% reduction in seizure frequency, and half had a 90% or greater reduction in seizure frequency. No adverse events were encountered. Comparison with published and registry patients revealed improved seizure control in the tuberous sclerosis complex patients. Comparison with the group undergoing seizure surgery demonstrated improved outcomes after surgery. Vagal nerve stimulation appears to be an effective and well-tolerated adjunctive therapy in patients with tuberous sclerosis complex and seizures refractory to medical therapy. Resective surgery has a better prospect for improved seizure control.
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