Authors :
Presenting Author: Ahda Jbarah, MD – Emory University/ Children's Healthcare of Atlanta
Ekta Shah, MD – Emory University/ Children's Healthcare of Atlanta
Kishore Vedala, MD – Emory University/ Children's Healthcare of Atlanta
Jenny Lin, MD – Children’s Healthcare of Atlanta
Goujon Zhang, MD – Emory University/ Children's Healthcare of Atlanta
Joshua Chern, MD – Childrens Healthcare of Atlanta
Corinne Oliver, MSN – Children’s Healthcare of Atlanta
Marleny Aquino, MD – Emory University/ Children's Healthcare of Atlanta
Hyoung Choi, MD – Emory University/ Children's Healthcare of Atlanta
Neal sankhla, MD – Emory University/ Children's Healthcare of Atlanta
Sonam Bhalla, MD – Emory University/ Children's Healthcare of Atlanta
Rationale:
Febrile Infection-Related Epilepsy Syndrome (FIRES) is a subtype of New-Onset Refractory Status Epilepticus (NORSE), characterized by new-onset, medically refractory seizures following a febrile illness. These seizures often require prolonged treatment with continuous infusions of antiseizure medications and are associated with significant morbidity and mortality. Vagus nerve stimulation (VNS) is an approved therapy for drug-resistant epilepsy and has been explored in the context of refractory and super-refractory status epilepticus (SRSE), including pediatric FIRES. Emerging reports suggest that early VNS implantation with rapid titration, starting stimulation early and increasing output current and optimizing duty cycle daily , may accelerate clinical response and reduce seizure burden. However, limited data exists on safety and outcomes in children.
Methods: We conducted a retrospective chart review of four pediatric patients (ages 5–13) with FIRES or NORSE admitted to Children’s Healthcare of Atlanta between 2023 and 2025. All patients received standard medical and immunotherapeutic management followed by VNS implantation in the acute or subacute phase. Rapid titration was initiated postoperatively. Clinical data, including demographics, neuroimaging, electroencephalography (EEG) findings, VNS parameters, seizure burden, and outcomes, were reviewed.
Results: The median lag between the time of presentation of SRSE and VNS implantation was 43 days (range 38–81). All three patients underwent daily rapid titration, one of them starting day of surgery and others on post operative day 1. Titration continued until the seizure burden significantly improved patient 1: current 2.25 mA, duty cycle 51%, patient 2: current 2.5 mA, duty cycle 58%, patient 3 current 2.75 ma, duty cycle 59 %, patient 4: current 2.75 ma, duty cycle 59% (Fig.1). This approach facilitated successful weaning off continuous anesthetic infusions . Three patients were discharged to rehabilitation with marked clinical improvement; one patient died due to multiorgan failure following prolonged hospitalization. One of the patients developed mild obstructive sleep apnea but otherwise no significant VNS-related complications were reported. At the most recent follow-up all three patients had significant seizure reduction (patient 1: 1-3 brief seizures daily, patient 2: 1-2 brief seizures per week, patient 4: 1-2 brief seizures every 3-4 months and no recurrence of status epilepticus.
Conclusions: This case series supports the growing evidence that VNS with rapid titration may be a safe and effective adjunctive treatment in pediatric patients with FIRES or NORSE. Our findings are in line with previous reports suggesting earlier response and potential role of VNS in acute management of prolonged super refractory status epilepticus. While promising, further research is required to define optimal timing, stimulation parameters, and long-term outcomes in this vulnerable population.
Funding: None.