Abstracts

Responsive Nerve Stimulation as a Treatment for Febrile Infection Related Epilepsy Syndrome (FIRES): A Case Report

Abstract number : 2.557
Submission category : 3. Neurophysiology / 3E. Brain Stimulation
Year : 2024
Submission ID : 1504
Source : www.aesnet.org
Presentation date : 12/8/2024 12:00:00 AM
Published date :

Authors :
Presenting Author: Marco Malaga, MD – Baylor College of Medicine

Ali Ahmad, MD – Baylor College of Medicine
Haroon Khilan, MD – Baylor College of Medicine
Mohammed Hasen, MD – Baylor College of Medicine
Alica Goldman, MD – Baylor College of Medicine

Rationale: New-onset refractory status epilepticus (NORSE) is a rare epileptic disorder characterized by sudden onset status epilepticus with no response to typical seizure treatments and no apparent cause, with FIRES being a subtype related to recent febrile infections. Even as the etiology of the disease is not completely understood, current treatment guidelines recommend immunomodulatory treatment as a first-line approach. However, neuromodulation is emerging as a promising complimentary measure. Here we report a patient with FIRES who underwent responsive neurostimulation (RNS) to the bilateral centro-median thalamic nuclei with improved seizure burden and neurocognition.

Methods: Case report of a 34 year old female with no significant past medical history who was transferred with refractory status epilepticus from an outside hospital, following five days of flu-like symptoms, fever and confusion. She was intubated and sedated. Upon arrival, a comprehensive diagnostic work-up including repeated cerebrospinal fluid (CSF) analysis, autoimmune and infectious panels, and imaging studies (MRI, PET-CT) was performed, all of which were unremarkable. Her treatment regimen was escalated to include multiple ASMs, high-dose methylprednisolone, plasmapheresis, intrathecal dexamethasone, and eventually Tocilizumab, following elevated CSF cytokines. Despite these interventions, her RSE persisted, prompting the placement of responsive neurostimulation (RNS) targeting the bilateral centro-median thalamic nuclei (CMT) on day 65 since seizure onset. RNS was rapidly uptitrated until day 73. Final settings were as follows: Current of 3.0 mA, frequency of 142.9 Hz, charge density of 1.5 microC/m2, and length of episodes of 30 seconds.

Results: Following the placement and titration of RNS, the patient showed gradual improvement. Within 11 days of RNS activation, the patient began following simple commands and responding to questions. She was subsequently discharged to a rehabilitation facility where she continued to make a remarkable cognitive and physical recovery. At three months post-discharge, RNS interrogation reported full seizure control with no electrographic evidence of seizures, and the patient was maintained on a tailored ASM regimen. Her functional status is 4 in the Glasgow Outcome Scale.

Conclusions: Our patient represents a case of significant improvement after RNS implantation in a patient with FIRES, despite the failure of immunomodulatory therapy. It adds to the growing body of evidence supporting the use of neuromodulation both for acute status and chronic seizure control.

Funding: Self-funded

Neurophysiology