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Pediatr Neurol. 2002 Nov;27(5):369-77.

Clinical study of catastrophic infantile epilepsy with focal seizures.

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  • 1Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan.


This study investigated clinico-electrical and etiologic characteristics of catastrophic infantile epilepsy with focal seizures developed in early infancy. The patients included 15 children who fulfilled the following criteria: seizure onset before 12 months of age, presence of daily focal or secondarily generalized seizures resistant to antiepileptic drugs for at least 3 months, and exclusion of Ohtahara and West syndromes. Patients were classified into three subgroups. Three patients demonstrated progressively deteriorating neurologic symptoms associated with progressive cerebral atrophy and multifocal seizure onset. Three other children were characterized by hemiparesis and exclusively lateralized seizure onset because of focal cortical dysplasia in the contralateral hemisphere. The remaining nine children did not demonstrate any rapidly progressive neurologic deterioration or increasing cerebral atrophy and exhibited multifocal seizure onset. At the last examinations, all except one patient demonstrated moderate to severe psychomotor retardation. Catastrophic infantile epilepsy with focal seizures tended to demonstrate multifocal seizure onset and a deleterious clinical course with numerous focal seizures regardless of etiology. Because migratory focal seizures appear to be common in these infants, we have to search for the underlying etiopathogenesis of these patients, including not only metabolic errors but also localized or lateralized structural abnormality.

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