Epilepsy surgery for pediatric patients with mild malformation of cortical development

Abstract

Purpose: The observation of mild malformation of cortical development (mMCD) has yet to have a major clinical impact due to the lack of clinical and research data. We characterized the clinical features, surgical outcomes, and postoperative seizure control patterns in pediatric patients with mMCD.

Methods: We examined 40 patients with isolated mMCD who underwent resective surgery during a 10-year period.

Results: The median age at seizure onset was 1.2 years, and the median age at surgery was 7.9 years. Twenty-seven patients (67.5%) presented with childhood-onset epileptic encephalopathy (21 Lennox-Gastaut syndrome, 6 West syndrome), and 13 patients (32.5%) presented with intractable focal epilepsy (10 extratemporal lesions, 3 temporal lesions). Twenty-one patients (52.5%) showed "suspected focal cortical malformation" on MRI, whereas 16 patients (40.0%) and 3 patients (7.5%) showed normal MRI findings or mild brain atrophy, respectively. The most common surgical procedures were two lobar resections (18 patients, 45.0%), followed by unilobar resections (12 patients, 30.0%) and resections exceeding two lobar boundaries (10 patients, 25.0%). As a final surgical outcome, 24 patients (60.0%) were ILAE Class 1-3. Discontinuation of all AEDs was possible for 36.8% of ILAE Class 1 patients. Regarding the seizure control pattern, fluctuating seizure control was observed most frequently (21 patients, 52.5%).

Conclusion: Our results suggest that mMCD is an important pathological finding in children related to a significant degree of epileptogenicity, and resective surgery can have positive outcomes. However, these patients showed unstable postoperative seizure control patterns with a high rate of late recurrence, suggesting difficulties in the surgical treatment of intractable epilepsy.