Vagus Nerve Stimulation (VNS) in Super Refractory New Onset Refractory Status Epilepticus (NORSE)

Abstract

The treatment protocol of status epilepticus has many associated toxicities so there is interest in alternate nonmedicinal therapies for managing New Onset Refractory Status Epilepticus (NORSE) patients. Vagus nerve stimulation (VNS) is an FDA-approved therapy for refractory epilepsy that has been shown to decrease the frequency and severity of seizures. We present the case of a patient with new-onset refractory status epilepticus (NORSE) whose seizures were successfully treated with vagus nerve stimulation. A 25-year-old male with no history of epilepsy or other neurological disorders presented with altered mental status and generalized tonic-clonic seizures following a two-week history of an upper respiratory tract infection. Lumbar puncture showed neutrophilic pleocytosis, and he was treated for bacterial and viral meningoencephalitis. In spite of treatment, his seizures began increasing in frequency. On day three, the patient entered status epilepticus (SE) refractory to intensive pharmacotherapy with maximal doses of valproate, levetiracetam, and propofol. On day four, SE remained refractory, so pentobarbital was introduced with targeted burst suppression pattern on electroencephalography (EEG). Patient continued to be refractory to these measures, so a vagus nerve stimulator (VNS) was implanted (day eight). Following VNS implantation, EEG demonstrated significant reduction of seizure activity and subsequent magnet swiping continued aborting electrographic seizures. No SE or electrographic seizures were reported for seventy-two hours, but few occasional discharges were reported. Seizures eventually recurred on day fourteen and the patient succumbed to his multiple comorbidities on day seventeen. Due to the efficacy of VNS in refractory epilepsy, there was interest in using it in refractory status epilepticus. Multiple case reports have described a benefit from implantation of VNS in the treatment of SE. The successful use of VNS to acutely terminate status epilepticus for seventy-two hours in this critically ill patient adds to current evidence that there is utility in using VNS for refractory status epilepticus.

International consensus recommendations for management of new onset refractory status epilepticus including febrile infection‐related epilepsy syndrome: Statements and supporting evidence

“…However, there was a consensus that in patients with focal onset seizures from unilateral or bilateral temporal regions, an evaluation for epilepsy surgery should be considered. Vagus nerve stimulation may be effective for postacute epilepsy (M = 7, MA = 7, MP = 7, LA = 75.0%, LD = 2.1%).There are very few available data to evaluate the efficacy and safety of vagus nerve stimulation (VNS) in the postacute phase of NORSE/FIRES. One case report of successful rapid titration of VNS parameters in a pediatric patient has been published, 108 and two case reports in adults also report positive effects in the acute 109 and postacute 110 phases. However, based on the possible anti‐inflammatory effect of VNS 111 and the long‐term use in several other forms of therapy‐refractory epilepsies, including RSE, 112–114 neuromodulation with VNS can also play a role in NORSE/FIRES and should be considered.…”

“…There are very few available data to evaluate the efficacy and safety of vagus nerve stimulation (VNS) in the postacute phase of NORSE/FIRES. One case report of successful rapid titration of VNS parameters in a pediatric patient has been published, 108 and two case reports in adults also report positive effects in the acute 109 and postacute 110 phases. However, based on the possible anti‐inflammatory effect of VNS 111 and the long‐term use in several other forms of therapy‐refractory epilepsies, including RSE, 112 , 113 , 114 neuromodulation with VNS can also play a role in NORSE/FIRES and should be considered.…”

Long‐term outcomes after NORSE: Treatment with vagus nerve stimulation

“…There was a clear relationship between VNS and cessation of RSE in only two cases 7,8 . A different report details a patient whose implantation was considered successful, seizures recurred, and the patient died 9 . In the other reports, it is unclear which of the multiple treatments or a combination thereof were responsible for ceasing the NORSE 4,10 .…”

Estado Epiléptico Refractario de Aparición Reciente (NORSE): Revisión y actualización

“…11,24,32 Existe un caso publicado en el que se suprimió por completo el EE durante 72 horas tras implantarse la ENV en un adulto con NORSE y, aunque las CE reaparecieron y el paciente acabó falleciendo, proporciona información sobre el posible uso de esta alternativa en la fase aguda del NORSE. 35 Otras alternativas de las que hay escasa evidencia de mejoría clínica en niños con FIRES incluyen la hipotermina terapéutica a 33ºC, como terapia adyuvante, y la infusión intravenosa de sulfato de magnesio. 19,32 Finalmente, la cirugía podría ser una opción para la epilepsia refractaria al tratamiento médico, en casos en que los estudios de neuroimagen, medicina nuclear y electroencefalográficos indiquen alguna anomalía focal limitada y unilateral asociada al NORSE.…”