Turkish Archives of Pediatrics
Original Article

Neurodevelopmental Findings and Epilepsy in Malformations of Cortical Development

1.

Department of Pediatrics, Marmara University School of Medicine, İstanbul, Turkey

2.

Department of Pediatric Neurology, Marmara University School of Medicine, Istanbul, Turkey

3.

Department of Child and Adolescent Psychiatry, Marmara University School of Medicine, İstanbul, Turkey

4.

Department of Radiology, Marmara University School of Medicine, İstanbul, Turkey

5.

Division of Pediatric Neurology, Department of Pediatrics, Marmara University School of Medicine, İstanbul, Turkey

Turk Arch Pediatr 2021; 56: 356-365
DOI: 10.5152/TurkArchPediatr.2021.20148
Read: 65 Downloads: 37 Published: 30 June 2021

Aim: The purpose of this study is to classify the malformations of cortical development in children according 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 with the diagnosis of malformation of cortical development, in Marmara University Pendik Research and Educational Hospital Department of Pediatric Neurology, were included in the study. Their epilepsy characteristics, electroencephalogram (EEG) findings, and prognosis were reported. Neurodevelopmental characteristics were evaluated by the Bayley Scales of Infant and Toddler Development (Bayley-III) for the ages of 0-42 months (n = 30); the Denver Developmental Screening Test-II (DDST-II) for ages 42 months-6 years (n = 11); and the Wechsler Intelligence Scales 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% tuberous sclerosis, 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, the
classification was 34.7% hemispheric/multilobar, 33.3% diffuse, and 32% focal. Seventy-five percent of the patients had a history of epilepsy, and 92% were resistant to treatment. The seizures started before the age of 12 months in diffuse malformations, and epileptic encephalopathy was 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 neurodevelopmental delay detected by DDST/Bayley-III; this was more common in patients with accompanying epilepsy (P < .05). As seen more commonly in patients with diffuse pathologies and intractable frequent 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 and neurodevelopmental prognosis can be predicted depending on the developmental process and type and extent of involvement. Patients should be followed up closely with EEG.

Cite this article as: Şah O, Türkdoğan D, Küçü k S, et al. Neurodevelopmental Findings and Epilepsy in Malformations of Cortical Development. Turk Arch Pediatr. 2021; 56(4): 356-365.

Files
EISSN 2757-6256