The Need for Cardiorespiratory Monitoring: A near SUDEP Case in a Patient with Craniosynostosis Syndrome and Temporal Lobe Encephaloceles
Abstract number :
1.5
Submission category :
18. Case Studies
Year :
2023
Submission ID :
1302
Source :
www.aesnet.org
Presentation date :
12/2/2023 12:00:00 AM
Published date :
Authors :
Presenting Author: Vivek Ganesh, PhD – Neurava Inc.
Jay Shah, PhD – Neurava Inc.; Joseph Ta, MD – Vanderbilt University Medical Center; Christopher Smith, MD – Vanderbilt University Medical Center; Kelly Lowen, CCRP – Vanderbilt University Medical Center; Trevor Meyer, BS – Neurava Inc.; Andre LaGrange, MD, PhD – Vanderbilt University Medical Center; Martin Gallagher, MD, PhD – Vanderbilt University Medical Center; William Nobis, MD, PhD – Vanderbilt University Medical Center
Rationale: Sudden unexpected death in epilepsy (SUDEP) is a fatal complication of epilepsy that kills approximately 4,000 Americans every year. Following the findings of the landmark MORTEMUS study, existing clinical data suggests that SUDEP is a cardiorespiratory collapse that occurs during or directly after a seizure. Monitoring for cardiorespiratory dysfunctions can therefore be critical in potentially preventing SUDEP.
Methods: We provide a case report of a patient with Apert-Crouzon syndrome and medically refractory temporal lobe epilepsy, with multiple temporal encephaloceles. Apert-Crouzon syndrome is a rare genetic disorder characterized by craniosynostosis, a condition where there is premature fusion of sutures of the skull bones. There is minimal evidence that patients with this syndrome are at increased risk for epilepsy. This subject is a young adult male, who was admitted to the epilepsy monitoring unit (EMU) as part of normal clinical care and was also enrolled in the Neurava arm wearable study.
Results: During surgical work-up, he was found to have multiple temporal encephalocoeles, mostly on the right. During the EMU admission, he experienced a severe generalized tonic-clonic seizure followed by near SUDEP, with an extended post-ictal apnea leading to asystole. The subject self-stabilized and was rushed to the ICU for further stabilization. Evidence of this cardiorespiratory collapse enabled the clinical care team to more aggressively pursue surgical resection due to the likely risk of continued life-threatening seizures. Patients with Apert-Crouzon syndrome have a small risk of ventricular septal defect or overriding aorta, but cardiology evaluation did not find any primary cardiac problems.
Conclusions: This case illuminates i) the potential importance of encephaloceles and their relationship to epilepsy in craniofacial abnormalities and ii) provides further evidence that post-ictal apnea may be an important mechanism of cardiorespiratory collapse seen in SUDEP. This cardiorespiratory event following a seizure was discovered because the patient was in the EMU for evaluation. Had these events occurred at home, no evidence would have been collected. Home monitoring and wearables that can identify and alert for seizures and associated cardiorespiratory dysfunctions can be a powerful tool to mitigate SUDEP risk by providing quantitative data to patients, physicians and caregivers.
Funding: This study was funded by Neurava Inc.
Case Studies