Sudden Unexpected Death in Epilepsy in a Patient with a Brain-Responsive Neurostimulation Device: A Case Report
Abstract number :
1.495
Submission category :
18. Case Studies
Year :
2023
Submission ID :
1297
Source :
www.aesnet.org
Presentation date :
12/2/2023 12:00:00 AM
Published date :
Authors :
First Author: Richard Wang, BS – New York Medical college
Presenting Author: Patricia McGoldrick, NP, MSN, MPA, FAES – Maria Fareri Children's Hospital, Valhalla, NY & Boston Childrens Health Physicians of NY &CT and NYMC
Patricia McGoldrick, Nurse Practitioner – Boston Children's Health Physicians; Steven Wolf, Medical Doctor – Boston Children's Health Physicians
Rationale: Sudden unexpected death in epilepsy (SUDEP) is the most common cause of epilepsy-related death. Although most witnessed SUDEPs follow seizures, underlying mechanisms are still uncertain. Studies of the pathophysiology of SUDEP have relied on experimental models and rare recordings of brain and body function at the time of the event. The brain-responsive neurostimulation (RNS) device from Neuropace is a therapy option for drug refractory epilepsy that can both record brain activity and preemptively terminate seizures; as such, patients that experience SUDEP while being treated with a RNS device can provide valuable information on neural electrophysiological activity at the time of the event.
Methods: We report here the history and electrocortigraphical (ECoG) recordings of a patient with drug refractory epilepsy treated with a RNS device that experienced SUDEP.
Results: A 14 year old female patient with history of Phelan-McDiarmid syndrome, autism and Lennox-Gastaut syndrome, with frequent drug-resistant seizures, had an RNS placed with electrode leads in the right orbitofrontal (OF) and right anterior cingulate regions, and later change of the OF lead to the centromedian thalamus. At 19 years of age, she was found by her parents unconscious and in cardiac arrest. Return of spontaneous circulation was achieved enroute to the hospital. Return of consciousness was not achieved and the patient died. Later analysis of electrical readings via RNS recordings at the time of death showed seizure onset in the right anterior cingulate lobe and persistence despite multiple RNS seizure termination attempts.
Conclusions: Here we present a case of SUDEP associated with onset of RNS-refractory seizures. Data on neural function at time of SUDEP is rare in the literature and does not always include a witnessed seizure at the time of or just before the death. Further use of devices capable of ECoG-monitoring may shed light into the mechanisms underlying SUDEP and possible interventions.
Funding: None
Case Studies