2023 American Academy of Neurology Abstract Website

Objective:

To describe demographics, clinical characteristics, presurgical data, surgical approach, and outcomes of epilepsy patients with encephaloceles at The University of Kansas Medical Center.

Background:

Drug-resistant epilepsy (DRE) comprises around 1/3 of epilepsy patients. Encephaloceles are increasingly recognized as a DRE cause with an unknown incidence and prevalence. It can take ≥15 years to identify encephaloceles in DRE patients, with severe implications for quality of life and costs of care. In many cases, surgical resection/repair can be curative.

Design/Methods:

IRB approval was obtained. Informed consent was not required from individual patients. Data were retrospectively abstracted from the electronic medical record. Data analysis was performed using REDCap.

Results:

We identified 48 patients with epilepsy and encephaloceles in our Epilepsy Clinic. Twenty-eight (58%) were female; the mean age was 48.6 years (range 25-85). The average time from epilepsy onset to encephalocele discovery was 7.5 years (SD 8.4). Thirty-three (75%) met criteria for DRE. Twenty-three (48%) had no epilepsy risk factors, but in patients that did, trauma was the most frequent (13 pts, 27%).

A lack of aura (14 patients, 29%) and psychic aura (déjà vu) (10 patients, 21%) were the two most frequently occurring auras. A slight majority had focal to bilateral tonic-clonic seizures (27 pts, 56%). Most encephaloceles were temporal (97%). A majority of encephaloceles were of unknown etiology (67%); 17% had associated venous sinus stenosis and 11% had trauma and IIH as possible etiologies, respectively.

Clinical, imaging, and electrophysiologic evaluation (scalp/invasive EEG) estimated that 68% of encephaloceles caused epilepsy. Nine patients underwent epilepsy surgery. No patient undergoing surgery died.

Conclusions:

Most of our patients with epilepsy and encephaloceles met criteria for DRE. Early encephalocele recognition in DRE patients, followed by a prompt pre-surgical evaluation may impact seizure control, quality of life, and the financial burden of DRE on individual patients and health care systems.