Encephalocele-associated temporal lobe refractory epilepsy: Report of two cases

Abstract

Encephaloceles are abnormal protrusions of brain tissue, meninges, and cerebrospinal fluid that result from defects in the skull base or cranial vault. These abnormalities can lead to seizure disorders and focal pharmacoresistant epilepsies. However, clinical suspicion and diagnosis are frequently delayed due to the significant challenges in interpreting initial imaging. Interpretation omissions can contribute to false diagnosis as non-lesional epilepsy in patients with encephalocele. This warrants appropriate imaging paradigms and careful interpretation. When clinical suspicion remains, further surgical exploration should be considered. Different treatment approaches, such as lesionectomy, temporal lobectomy, or invasive studies such as stereoelectroencephalography, may be employed. We describe two cases of adult-onset epilepsy associated with encephaloceles: a 28-year-old woman with drug-resistant epilepsy secondary to a right temporal encephalocele that was not initially diagnosed through neuroimaging, and a 43-year-old woman who presented with de novo focal status epilepticus attributed to a left temporal encephalocele, diagnosed during surgical exploration for a misdiagnosed neoplasm. Both cases were successfully treated surgically, with one-year follow-up free of new seizures.