The epileptic characteristics and their differences in patients with
porencephaly and
schizencephaly were, respectively, evaluated. Eleven patients with
porencephaly and eight patients with
schizencephaly were retrospectively enrolled in this study. Five of the six patients with extensive
porencephaly and all five patients with open-lip
schizencephaly had been suffering from various types of epileptic
seizures. Three patients with extensive
porencephaly and all five patients with open-lip
schizencephaly had presented with early onset
seizures before 9 months of age. Two patients with extensive
porencephaly and three patients with open-lip
schizencephaly had presented with
West syndrome. These two groups of patients with epileptic
seizures showed
generalized epilepsy or
generalized epilepsy with unilateral dominancy at the onset, and then developed
localization-related epilepsy or unilateral
seizures with increasing age. The epileptic paroxysms showed multifocal independent spikes, which were not always localized in the defect or cleft sites at the last examination. Polytherapy or synergistic combinations were eventually introduced for these intractable
seizures in both groups for patients without any evidence of efficacy. In the
porencephaly patients, four of five patients achieved good seizure control with appropriate monotherapy or two-
drug therapy including
valproate. All five patients with
schizencephaly had been treated by polytherapy, and three of them had persistent intractable
seizures in spite of trying rational monotherapy or two-
drug therapy. The epileptic intractability associated with open-lip
schizencephaly might be related to the epileptogenesis of these extensive and widespread defective lesions, which were commonly associated with
cortical dysplasia. A trial of rational monotherapy or two-
drug therapy may be effective, rather than larger-number polytherapy in many cases, more in
porencephaly than
schizencephaly.