Epilepsy in paediatric patients with schizencephaly

Introduction: Schizencephaly is one of the rare congenital defects of the central nervous system (CNS), known as neuronal migration disorders. The etiology of schizencecephaly is unequivocal. Established etiologies include in-utero infections (cytomegalovirus and herpes simplex virus, HSV type I), toxic abuse (cocaine, alcohol), as well as drug use (warfarin).

Objectives: he aim of the study was to analyze the clinical presentation of schizencephaly with particular consideration of the course of epilepsy in paediatric patients.

Material and methods: The study group consisted of 38 children with schizencephaly (20 of them had seizure) and was retrospectively assessed. Data were analyzed using SAS version 9.4. U Mann-Whitney and χ 2 tests and logistic regression analysis were used in statistical analyses.

Results: Epilepsy was the most frequent in bilateral type II schizencephaly (p=0.033). In logistic regression analysis, the presence of bilateral open schizencephaly significantly increased the risk of seizures (OR=11.67; 95%CI 2.44-55.83; p=0.002). Drug-resistant epilepsy was observed in 9 children (45% of the children with epilepsy). Prevalence of both epilepsy and drug-resistant epilepsy in schizencephaly did not significantly depend on gender, stage of development, type or localization of schizencephaly, and other coexisting CNS defects or clinical presentation of schizencephaly at follow-up in the study group of patients.

Conclusions: The bilateral type of schizencephaly was identified as an independent risk factor for epilepsy in the analyzed children.

Keywords: bilateral; central nervous system malformation; developmental delay; epilepsy; schizencephaly; unilateral.