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Prolonged epileptic blindness in an infant associated with cortical dysplasia
1Child Neurology Unit, Rambam Medical Center, Haifa, Israel. e_shahar@rambam.health.gov.il
Insights
Surgical resection of congenital cortical dysplasia causing intractable epilepsy in an infant led to seizure cessation and vision recovery. Early EEG is crucial for diagnosing concealed epileptic activity in infants with suspected delayed visual maturation.
Area of Science:
- Pediatric Neurology
- Epileptology
- Developmental Neuroscience
Background:
- Congenital cortical dysplasia can manifest as intractable epilepsy in infants.
- Status epilepticus amauroticus and focal motor seizures present significant diagnostic and therapeutic challenges.
Observation:
- A female infant presented with severe epilepsy and visual impairment attributed to congenital cortical dysplasia.
- Electroencephalography (EEG) revealed epileptiform discharges in the right parieto-temporal and occipital regions.
- High-dose antiepileptic drug combinations failed to control the seizures.
Findings:
- Cortical resection of the identified epileptic focus resulted in complete cessation of seizures and restoration of vision within 48 hours.
- Histological analysis confirmed cortical dysplasia in the resected tissue.
- Long-term follow-up showed sustained seizure control with medication and intact cognition and speech.
Implications:
- Surgical intervention for focal epilepsy secondary to cortical dysplasia can achieve excellent outcomes, including visual recovery.
- EEG is essential for identifying occult epileptic activity in infants presenting with delayed visual maturation.
- This case highlights the importance of a multidisciplinary approach in managing pediatric epilepsy with cortical malformations.
Abstract:
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.