Intractable complex partial seizures associated with occult temporal lobe encephalocele and meningoangiomatosis: a case report.

Occult congenital temporal lobe encephalocele has rarely been reported in association with medically intractable complex partial seizures. The four previously reported cases were unsuspected preoperatively. We present the case of an 18-year-old woman with intractable complex partial seizures since age 13. Seizure onset was electrically localized to the right temporal lobe. Preoperative neuroimaging studies revealed a middle fossa defect and inferior herniation of the right temporal lobe. Pathologic examination of the resected encephalocele revealed prominent features of meningoangiomatosis. We believe this to be the first case of temporal lobe encephalocele and epilepsy to be diagnosed preoperatively, and the first case also to be associated with meningoangiomatosis. The relevant literature on meningoangiomatosis and on temporal lobe encephalocele as a cause of epilepsy is reviewed.