Abstracts

Rationale: Super- refractory status epilepticus (SRSE) is defined as persistent seizures or recurrence of seizures 24 hours after anesthetic anti-seizure agents are started. There are a few reports on the use of Vagal Nerve Stimulation (VNS) in refractory status epilepticus in children (1,2). We asked the question whether VNS can be used as an adjunctive therapy in SRSE. We report on a 10-month-old boy with hypoxic-ischemic encephalopathy s/p "partial" right temporal lobe resection, a 28-month-old boy with a nearly homoplasmic m.11778G>A mutation and progressive mitochondrial encephalopathy, and a 6-year-old boy with left mesial temporal sclerosis, who was unable to undergo epilepsy surgery. They were in SRSE and were implanted with VNS after either surgical or medication failure. Methods: Retrospective chart review of EEGs to confirm electroclinical and electrographic seizures, hospitalization, and follow-up records were done. The primary endpoint was reduction in number of anti-seizure medications. The secondary endpoint was reduction in the number of anti-seizure medications. Results: At 2 year follow-up, there was 100% seizure reduction in patients 1 and 3, and 50% reduction in patient 2 who has the progressive mitochondrial encephalopathy and is currently undergoing epilepsy surgery evaluation. There was a mean reduction of 2 anti-seizure medications at 2-year follow-up. The total number of medications decreased from 4 to 2, 8 to 5, and 6 to 5, respectively. Conclusions: The poor response of patient 2 is likely due to his progressive mitochondrial disease. Arthur (3) and colleagues have reported no response to VNS therapy in 5 patients with mitochondrial disease. The good response of patients 1 and 3 point to a potential role for VNS as an adjunctive therapy in patients who failed surgical intervention or anti-seizure medications as seen at 2 year follow-up. The lag time in the response to VNS makes it a poor option in hyperacute cases where early cessation of seizures is the goal. For patients who are epilepsy surgery candidates, epilepsy surgery should remain as the primary treatment option. 1De Herdt V et al, European Paediatric Neurology Society, 2009 (13); 286-289 2Winston K et al, Pediatric Neurosurgery, 2001; 34: 190-192 3Arthur TM et al, Mitochondrion, 2007; 7(4): 279-283 Funding: NONE