Prolonged epileptic blindness in an infant associated with cortical dysplasia

E Shahar1, P A Hwang

  • 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.

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