title: Clinical study of catastrophic infantile epilepsy with focal seizures.
abstract: 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.
authors.affiliation: Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Kayano
authors.initials: K
authors.lastname: Ishii
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Oguni
authors.forename: Kitami
authors.initials: K
authors.lastname: Hayashi
authors.forename: Seigo
authors.initials: S
authors.lastname: Shirakawa
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Itoh
authors.forename: Makiko
authors.initials: M
authors.lastname: Osawa
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use; D001284:Atrophy / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D002675:Child, Preschool; D003937:Diagnosis, Differential; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000150:complications / Q000175:diagnosis; D018450:Disease Progression; D004569:Electroencephalography; D004828:Epilepsies, Partial / Q000145:classification / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D038421:Hemispherectomy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000145:classification / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D010291:Paresis / Q000150:complications / Q000175:diagnosis; D011379:Prognosis; D011596:Psychomotor Disorders / Q000175:diagnosis / Q000209:etiology; D013036:Spasms, Infantile / Q000150:complications / Q000175:diagnosis; D014057:Tomography, X-Ray Computed
chemical_list: D000927:Anticonvulsants