Encephalocele-Associated Drug Resistant Epilepsy of Adult-Onset: Diagnosis, Management, and Outcomes

ABSTRACT Epileptogenic encephaloceles, most frequently located in the temporal lobe, are a known lesional cause of focal epilepsy. Data are limited regarding diagnosis, management, and outcomes of patients with epilepsy in the setting of an encephalocele, as the literature is mostly comprised of case reports, case series, and retrospective studies. We conducted a broad literature review for manuscripts related to encephaloceles and epilepsy regardless of level of evidence. Hence, this manuscript provides a summary of all available literature related to the topic. Thirty-six scientific reports that fulfilled our inclusion criteria were reviewed. The majority of reported cases presented with focal impaired awareness seizures and/or generalized tonic-clonic seizures. Although most of the encephaloceles were located in the temporal lobe, we found five cases of extra-temporal encephaloceles causing epilepsy. A greater proportion of cases that underwent either lesionectomy or lobectomy were seizure free at time of follow-up. In the temporal lobe, there is no clear consensus on the appropriate management for epileptic encephaloceles and further studies are warranted to understand the associated factors and long-term outcomes associated to epilepsy secondary to encephaloceles. Published data suggests that these cases could be potentially be manageable with surgical procedures including lesionectomy or lobectomy. Additionally, due to data suggesting similar results between procedures, a more conservative surgery with lesionectomy and defect repair rather than a lobectomy may potentially have lower surgical risks while having similar seizure freedom.