Childhood epilepsies are often associated with a variety of brain anomalies. Among epileptic syndromes of childhood, catastrophic epilepsies, including West and Lennox-Gastaut syndromes, are associated with the broadest spectrum of pathoanatomic abnormalities (180), including neuronal migration disorders and small foci of neuronal necrosis. Therefore, pre- and perinatal brain alterations become a condition sine qua non for the development of experimental seizures in the immature brain. The long-term goal of these studies is to induce a form of brain injury that would constitute a basis for the development of spontaneous seizures. The model, therefore, should demonstrate structural abnormalities and subsequent increased epileptogenicity in response to environmental stimuli, as well as spontaneous seizures. Until now, only few attempts have been made to approach developmental seizures in the presence of morphologic brain anomalies. How does the presence of brain anomalies or malformations lead to the development of epilepsy? Currently, data exist in four models of experimentally induced malformations: methylazoxymethanol-induced migration disorder, neocortical freezing lesions, neuronal migration disorder induced by irradiation in utero, and the double cortex mutation.
Was this article helpful?