Abstracts

Rationale: Treatment of Dravet syndrome is challenging and frequently disappointing in early childhood. Non-pharmacologic treatments for epilepsy including ketogenic diet, corpus callosotomy and Vagal Nerve Stimulation are considered and used for management of medically refractory epilepsy. In this series we report on the efficacy and tolerability of Vagal Nerve Stimulation in 6 children with Dravet syndrome. Methods: A retrospective chart review of patients with Dravet syndrome implanted with Vagal Nerve Stimulator (VNS) for treatment of intractable epilepsy between 2005 and 2012 at tertiary care academic epilepsy center was performed. Efficacy for seizure control, alertness, cognition was evaluated. Other information sought included number of anti-epileptic medications added after VNS implantation as a measure of efficacy of VNS. Results: 6 children with Dravet syndrome received VNS for intractable epilepsy. All were girls. Age at VNS implantation ranged from 2-7 years. Mean age was 5.3 years and median age was 6.5 years. Average duration of treatment with VNS ranged from 0.5 to 11 years with an average of 4.9 years. When compared to baseline 3 of 6 children had a 50-75% %reduction in seizures. 1 child had no change in seizure frequency but had decreased duration of seizures which resulted in reduced use of rectal valium and emergency room visits. 2 children had no significant improvement in seizures after VNS. In 1 child VNS was turned off around the time of anticipated battery replacement. Complex partial seizures recurred and VNS was turned back on. Battery was replaced after 8-9 years in 2 patients. In 1 child significant improvement in alertness and interaction was observed after VNS. The device was well tolerated in all patients without significant complications. Conclusions: 1. VNS is well tolerated in young children with Dravet syndrome. 2. 4 of 6 children had a significant improvement in seizure control . 3. 1 child had an improvement in cognitive function in addition to reduction in seizures. 4. Response to VNS in children with Dravet syndrome is similar to that reported in other intractable childhood epilepsies such as Lennox- Gastaut syndrome. 5. VNS should be considered as an option for children with Dravet syndrome who have medically intractable epilepsy and/or cannot tolerate medications.