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

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, because the literature mostly comprises case reports, case series, and retrospective studies. We conducted a broad literature review for articles related to encephaloceles and epilepsy regardless of level of evidence. Hence, this review provides a summary of all available literature related to the topic. Thirty-six scientific reports that fulfilled our inclusion criteria were reviewed. Most reported patients 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 5 cases of extratemporal encephaloceles causing epilepsy. More patients who 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 with epilepsy secondary to encephaloceles. Reported data suggest that these patients could be manageable with surgical procedures including lesionectomy or lobectomy. In addition, because of data suggesting similar results between procedures, a more conservative surgery with lesionectomy and defect repair rather than a lobectomy may have lower surgical risks and similar seizure freedom.