Abstracts

Rationale: Objective:To describe the clinical efficacy of epilepsy surgery with responsive neurostimulation, corticosteroids, and intravenous immunoglobulin (IVIG) for the treatment of chronic refractory epilepsy resulting from autoimmune encephalitis. Background:Autoimmune encephalitis frequently presents with seizures in the acute setting. Seizures are often refractory to anti-seizure medications but have been shown to be responsive to immunomodulatory therapies. Current standard practice for treatment of autoimmune encephalitis involves treatment with high dose corticosteroids, IVIG, or plasmapheresis (PLEX) as first-line immunotherapy, and consideration of rituximab and cyclophosphamide as second-line agents. A subset of patients continue to have chronic refractory epilepsy for years after the acute illness. Optimal treatment for these patients has not been determined. Furthermore, the efficacy of responsive neurostimulation or immunotherapy in chronic refractory epilepsy due to autoimmune encephalitis has not been established. Methods: Retrospective chart review was performed in a patient with autoimmune encephalitis referred to the epilepsy center at a tertiary care center for chronic refractory epilepsy. Electrocorticography was reviewed to determine change in seizure frequency. Results: We report the clinical course of a patient with new onset refractory status epilepticus in the setting of autoimmune encephalitis. The patient underwent comprehensive evaluation and standard management with anti-seizure medications, followed by immunotherapy in the acute setting. Five years after his acute presentation, he presented to our epilepsy center with continued frequent seizures that were refractory to four anti-seizure medications at therapeutic doses. Continuous video EEG monitoring revealed independent bilateral temporal lobe seizures. A responsive neurostimulation system (RNS) was placed and titrated over eight months without significant change in seizure frequency. Due to continued frequent seizures despite anti-seizure medications and surgical management, he was given a trial of corticosteroids and IVIG. Electrocorticography was reviewed on the patient data management system (PDMS) and used as an objective surrogate measure of seizure frequency. Despite the addition of immunotherapy to his treatment regimen, the patient experienced no clinical or electrographic change in seizure frequency. Conclusions: There was no clinical or electrographic change in the frequency of seizures in a patient with chronic refractory epilepsy due to autoimmune encephalitis with multiple antiepileptic mediations, epilepsy surgery with the RNS and immunotherapy. This case does not support the use of immunotherapy for treatment of chronic refractory epilepsy following autoimmune encephalitis, and the patient is a responsive neurostimulation non-responder. Funding: No funding