Schizencephaly: Case Report

Abstract

Problem statement: Whereas schizencephaly is a malformation of cortical development which exact cause remains unknown, is almost entirely characterized by clefts extending from the pial surface to ependymal surface and could be unilateral or bilateral. Oftentimes, it also occurs in association with other abnormalities. Over the years, two types were described in literature, lips closed and open. Therefore, our intention is describe a case of schizencephaly monitored by the Neurology Service of Nova Iguacu University Hospital. Approach: A 29-year old single male student, presenting clinical history of epilepsy since he was 23 years old (partial seizures with secondary generalization) showed a global delay in neuropsychomotor development. Results: The patient has been admitted in the emergency department and his seizures were controlled with IV diazepan, followed by phenobarbital and phenotoyn. CT scan has revealed a cortical maldevelopment described as bilateral schizencephaly. After these procedures, he was released six days later, seizure-free, using carbamazepine and phenotyn. Conclusion: Is indeed that prognosis for individuals with schizencephaly varies depending on the size of the clefts and the neurological deficit degree. In general, treatment for individuals with schizencephaly usually consists of physical therapy, occupational therapy, treatment for seizures and, in a few cases complicated by hydrocephalus, a shunt is required.