Closed-Lip Schizencephaly, as a cause of refractory epilepsy: Case Report.

Abstract

Background: Schizencephaly is a congenital disorder of the central nervous system characterized by clefts that connect the subarachnoid space with the lateral ventricles. There are 2 types, closed lip (type 1), in which the edges of the cleft come into contact, and open lip (type 2), in which the lips are significantly separated by cerebrospinal fluid, this being the one with the worst prognosis. . It is a rare disease belonging to neuronal migration disorders, with a prevalence of 1.54/100,000 live births. Clinical manifestations can range from an asymptomatic spectrum to repeated seizures with severe neuronal alteration. The imaging method of choice for diagnosing this pathology is magnetic resonance imaging. Case description: We present a case of a female minor infant patient with a personal pathological history of repeated epileptic seizures, who was admitted for presenting generalized tonic-clonic episodes, loss of alertness and relaxation of sphincters, presenting hypotonia in all 4 extremities, without evident phenotypic alterations and with psychomotor delay. Due to this, she underwent resonance imaging studies that allowed the diagnosis of closed lip schizencephaly (type 1) plus epileptic seizures that were difficult to manage. Conclusion: Neuronal migration disorders such as schizencephaly should be suspected and investigated in all patients with epilepsies refractory to drug treatment.