Abstract
In the acute phase of Febrile infection-related epilepsy syndrome (FIRES), super-refractory status epilepticus (SE) is a common clinical symptom. This research aimed to study effectiveness of vagus nerve stimulation (VNS) and explore a rapid parameter programming protocol suitable for VNS in patients with the acute phase of FIRES-related super-refractory SE. The children with acute phase of FIRES-related prolonged super-refractory SE in ICU were enrolled and divided into VNS and control group based on whether the patient underwent VNS. A standardized rapid programming protocol of stimulation parameters was used in patients with VNS. The patients' demographic data, treatments method, time of SE termination, percentage of seizure freedom, mortality, activities of daily living, and Glasgow Outcome Scale (GOS) were collected and analyzed. Sixty patients were enrolled, and 30 in VNS group underwent VNS with rapid parameter programming protocol. A significant difference was found in their duration of SE after enrollment between groups. VNS was the exclusive influence factor for post-enrollment mortality of all patients and seizure freedom in surviving patients. Patients without VNS presented a significantly higher probability of death (HR = 3.46) and recurrent seizures (HR = 7.75) compared to those with VNS. A significant difference was also observed in the percentage of patients with ADL scores >60 between the two groups at follow-up. No obvious complication was reported for the surgery of VNS and during the electrical stimulation process. VNS with rapid parameters programming protocol was a safe approach for treating super-refractory SE in the acute phase of FIRES. It can significantly reduced the duration of SE, mortality, and recurrent seizure.