Neurodevelopmental Findings and Epilepsy in Malformations of Cortical Development

Özet

Aim: The purpose of this study is to classify the malformations of cortical development in childrenaccording to the embryological formation, localization, and neurodevelopmental findings.Seizure/epilepsy and electrophysiological findings have also been compared.Material and Methods: Seventy-five children (age: 1 month-16.5 years; 56% male) followed withthe diagnosis of malformation of cortical development, in Marmara University Pendik Researchand Educational Hospital Department of Pediatric Neurology, were included in the study. Theirepilepsy characteristics, electroencephalogram (EEG) findings, and prognosis were reported.Neurodevelopmental characteristics were evaluated by the Bayley Scales of Infant and ToddlerDevelopment (Bayley-III) for the ages of 0-42 months (n = 30); the Denver DevelopmentalScreening Test-II (DDST-II) for ages 42 months-6 years (n = 11); and the Wechsler IntelligenceScales for Children (WISC-R), used for children 6 years and older (n = 34).Results: The patients were classified as 44% premigrational (14.6% microcephaly, 24% tuberoussclerosis, 2.7% focal cortical dysplasia, 1.3% hemimegalencephaly, and 1.3% diffuse cortical dysgenesis);17.3% migrational (14.6% lissencephaly, 2.7% heterotopia); and 38.6% postmigrational(14.6% schizencephaly, 24% polymicrogyria) developmentally. According to involved area, theclassification was 34.7% hemispheric/multilobar, 33.3% diffuse, and 32% focal. Seventy-five percentof the patients had a history of epilepsy, and 92% were resistant to treatment. The seizuresstarted before the age of 12 months in diffuse malformations, and epileptic encephalopathywas more common in microcephaly with a rate of 80% and lissencephaly with a rate of 54.5%in the first EEGs. Ninety-five percent of patients had at least one level of neurodevelopmentaldelay detected by DDST/Bayley-III; this was more common in patients with accompanyingepilepsy (P < .05). As seen more commonly in patients with diffuse pathologies and intractablefrequent seizures, mental retardation was detected by WISC-R in 64.5% of patients (P < .05).Conclusion: In cases with cortical developmental malformation, epilepsy/EEG features andneurodevelopmental prognosis can be predicted depending on the developmental process andtype and extent of involvement. Patients should be followed up closely with EEG.