Refractory Status Epilepticus in Genetic Epilepsy—Is Vagus Nerve Stimulation an Option?

Nicola Specchio,Alessandro Ferretti,Giusy Carfì Pavia,Carlo Efisio Marras,Marina Trivisano,Costanza Calabrese,Federico Vigevano,Luca De Palma,Nicola Pietrafusa,Alessandro De Benedictis

doi:10.3389/fneur.2020.00443

Abstract

Refractory and super-refractory status epilepticus (RSE, SRSE) are severe conditions that can have long-term neurological consequences with high morbidity and mortality rates. The usefulness of vagus nerve-stimulation (VNS) implantation during RSE has been documented by anecdotal cases and in systematic reviews; however, the use of VNS in RSE has not been widely adopted. We successfully implanted VNS in two patients with genetic epilepsy admitted to hospital for SRSE; detailed descriptions of the clinical findings and VNS parameters are provided. Our patients were implanted 25 and 58 days after status epilepticus (SE) onset, and a stable remission of SE was observed from the seventh and tenth day after VNS implantation, respectively, without change in anti-seizure medication. We used a fast ramp-up of stimulation without evident side effects. Our results support the consideration of VNS implantation as a safe and effective adjunctive treatment for SRSE.

Highlights

Refractory and super-refractory status epilepticus (RSE, SRSE) are severe conditions that can have long-term consequences, including alteration of neuronal networks, neuronal injury, and high morbidity and mortality rates [1, 2]
The usefulness of vagus nerve-stimulation (VNS) implantation during RSE has been documented by anecdotal cases and in systematic reviews [3,4,5,6]
Previous evidence shows the median duration of RSE/SRSE pre- and post-VNS implantation to be 18 and 8 days, respectively [4]

Summary

INTRODUCTION

Refractory and super-refractory status epilepticus (RSE, SRSE) are severe conditions that can have long-term consequences, including alteration of neuronal networks, neuronal injury, and high morbidity and mortality rates [1, 2]. AE, adverse event; LCM, lacosamide; IgIV, intravenous immune globulin; KD, ketogenic diet; KET, ketamine; LEV, levetiracetam; MDZ, midazolam; PB, phenobarbital; PER, perampanel; SRSE, super-refractory status epilepticus; TPM, topiramate; VNS, vagus nerve stimulation; VPA, valproate This was a female patient who was aged 16 years at the time of our observations. Seizures occurred weekly from ages 12 to 16, with some intermittent reduction in seizure frequency when a new ASM was added She came to our Department of Neuroscience with frequent myoclonic jerks involving the left side of the body, turning to super refractory myoclonic status. Seizures were resistant to multiple ASMs (pyridoxin, phenobarbital, carbamazepine, phenytoin, clonazepam, and topiramate) She continued to present focal to bilateral tonic-clonic seizures every 4–8 days. Pediatric acute respiratory distress syndrome (P-ARDS) at the age of 3

DISCUSSION

Findings

ETHICS STATEMENT