Prolonged epileptic blindness in an infant associated with cortical dysplasia.
We report a female infant with status epilepticus amauroticus and intractable focal motor seizures associated with congenital cortical dysplasia. EEG demonstrated persistent epileptiform discharges over the right parieto-temporal regions extending to occipital areas. She required cortical resection of the epileptic zone at age 8 months given failure of very high dose antiepileptic combinations. Histological analysis of a sample of cortex resected from the right central parieto-temporal region, identified by electrocorticography as the focus of epileptic activity, showed cortical dysplasia. The seizures ceased and the infant gained full vision after 48 hours. During an 8-year follow-up period she has had a few short-lived seizures, currently controlled with carbamazepine and vigabatrin. Her cognition and speech are intact. This case demonstrates that: (1) resection of a central temporo-parietal focus, which may have spread to the occipital regions, may result in complete visual recovery and cessation of seizures; and (2) EEG should be considered in every infant with alleged delayed visual maturation, to rule out concealed epileptic activity.
Shahar
E
E
Child Neurology Unit, Rambam Medical Center, Haifa, Israel. e_shahar@rambam.health.gov.il
Hwang
P A
PA
D001766:Blindness / Q000209:etiology*; D002540:Cerebral Cortex / Q000473:pathology*; D002657:Child Development; D003071:Cognition; D003937:Diagnosis, Differential; D004569:Electroencephalography; D004827:Epilepsy / Q000150:complications* / Q000209:etiology*; D005260:Female; D006801:Humans; D007223:Infant