The differences in epileptic characteristics in patients with porencephaly and schizencephaly.
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.
Department of Pediatrics and Child Neurology, Oita University Faculty of Medicine, Yufu, Oita, Japan. ntkymiki@oita-u.ac.jp
Miki
M
Shimizu
Tomoki
T
Maeda
Tatsuro
T
Izumi
D000293:Adolescent; D000328:Adult; D001927:Brain Diseases / Q000150:complications / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D002648:Child; D024141:Collagen Type IV / Q000172:deficiency; D004338:Drug Combinations; D004569:Electroencephalography; D004827:Epilepsy / Q000188:drug therapy / Q000209:etiology / Q000473:pathology / Q000503:physiopathology*; D005260:Female; D006429:Hemiplegia / Q000150:complications / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D008297:Male; D054220:Malformations of Cortical Development / Q000150:complications / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D065708:Porencephaly; D012189:Retrospective Studies; D055815:Young Adult
D024141:Collagen Type IV; D004338:Drug Combinations