Schizencephaly: Correlation between clinical and neuroimaging features

Schizencephaly is an uncommon congenital brain malformation. We report our experience of 13 patients with schizencephaly and evaluate the clinical, neuroradiologic, electroencephalographic (EEG), and nosological features. Of these 13 patients, 8 were unilateral forms, 5 were bilateral forms and 11 were open-lip type schizencephaly. One patient was proven to have cytomegalovirus (CMV) infection. The clinical features and neurodevelopmental outcomes are variable. Although seizure developed in 9 patients (5 patients from unilateral and 4 from bilateral forms), the severity of epilepsy was not totally related to the degree of malformations. The neurodevelopmental outcome depended on the extent of schizencephaly as well as the seizure control. Those with bilateral forms and intractable seizures had the worst outcome. Other central nervous system (CNS) anomalies were observed in 11 patients. Six out of 11 patients had focal cortical dysplasia. We conclude that children with schizencephaly usually have variable neurological impairment. Earlier diagnosis of schizencephaly and related CNS malformation with neuroimaging is helpful in predicting the neurodevelopmental outcomes in these patients.