Refractory epilepsy and encephalocele: Lesionectomy or tailored surgery?

We read with great interest the paper entitled ‘‘Tailored surgery for drug-resistant epilepsy due to temporal pole encephalocele and microdysgenesis’’ by Giulioni et al. The authors describe two cases of encephalocele associated with cortical microdysgenesis and assert the necessity to consider an extensive surgical resection in subjects with small encephaloceles, due to the possible concomitance of malformations of cortical development. We recently observed two patients that may represent two opposite situations of patients. The first subject is a woman who experienced a tonic– clonic seizure during sleep at age 14 years, and was started with carbamazepine (CBZ) up to 1200 mg/day. No seizures appeared for 8 years. At age 22, she started to experience brief, weekly complex partial seizures, described as loss of consciousness, gestural and bipedal automatisms, followed by confusional status. Seizures persisted despite treatment with numerous antiepileptic drugs in various combinations. At age 35, when she came to our observation, physical and neurologic examinations were normal. Video-EEG monitoring allowed recording a nocturnal seizure originating from wide area of the right frontal lobe. MRI showed a small encephalocele associated with a linear cortical hyperintensity in T2-weighted sequences, localized in right anterior frontal region (Fig. 1A). Neuropsychological examination showed impairment in visuospatial memory, constructive apraxia and difficulties in planning actions, consistent with a right frontotemporal impairment. At age 36, she underwent lesionectomy and resection of adjacent cortex (Fig. 1B). Histologic examination showed encephalocele and an associated type IB focal cortical dysplasia (FCD), extending beyond the resection margin. Surgery was uncomplicated, without any additional post-operative neurologic or neuropsychological deficits, but seizure frequency was not modified (Engel class IV) at 5 years of follow-up. Patient refused further surgical interventions. The second patient is a 20-year-old man who came to our observation at age 14 for rare seizures characterized by déjà-vu, dreaming state and sometimes oral automatisms. Treatment with carbamazepine resulted in seizure freedom for 14 months, then he started experiencing weekly complex partial seizures with cephalic aura, loss of consciousness, oral and gestural automatisms, dystonia and clonic movements of right upper limb, followed by confusion state. Adjunctive treatment with topiramate and, subsequently, lacosamide did not modify seizure frequency. Standard brain MRI was deemed normal. At age 20 he was evaluated for epilepsy surgery. Prolonged video-EEG monitoring allowed detecting three seizures originating from left temporal lobe. High-resolution CT scan (Fig. 1B) showed a small bony defect in left mesial portion of middle cranial fossa. MRI targeted at temporal pole (Fig. 1C) evidenced a tiny encephalocele