Hemispherectomy in early infantile epileptic encephalopathy
Lorie Hamiwka1, Michael Duchowny, Israel Alfonso
1Department of Neurology. Miami Children's Hospital, 3200 SW 60th Court, Miami, FL 33155, USA.
Insights
Early infantile epileptic encephalopathy in children with hemispheric dysplasia can be successfully treated with functional hemispherectomy. This surgical intervention leads to seizure freedom and improved development, highlighting the benefits of early surgical treatment.
Area of Science:
- Pediatric Neurology
- Developmental Neuroscience
- Surgical Neurology
Background:
- Early infantile epileptic encephalopathy (EIEE) presents a significant challenge in pediatric neurology.
- Hemispheric dysplasia, a form of cortical malformation, is a known cause of severe epilepsy in infants.
- Identifying effective treatment strategies for EIEE is crucial for improving long-term outcomes.
Observation:
- Two children diagnosed with EIEE and hemispheric dysplasia underwent surgical evaluation.
- Focal epileptic patterns and ipsilateral hyperperfusion in the affected hemisphere were identified.
- Electrocorticography confirmed near-continuous spike and slow-wave discharges, indicating severe epileptiform activity.
Findings:
- Histopathology confirmed Taylor-type cortical dysplasia as the underlying cause.
- Functional hemispherectomy was performed, removing the epileptogenic and dysplastic brain tissue.
- Both patients achieved complete seizure control post-surgery with monotherapy antiepileptic drugs.
Implications:
- Early surgical intervention, such as functional hemispherectomy, can be highly effective for EIEE caused by unilateral cortical malformations.
- This approach offers a potential pathway to seizure freedom and improved developmental trajectories in affected children.
- The findings support considering surgical options proactively in select cases of infantile epilepsy with focal cortical dysplasia.
Abstract:
The authors report the successful surgical treatment of early infantile epileptic encephalopathy and suppression burst in 2 children with hemispheric dysplasia. At surgical evaluation, focal epileptic patterns were recorded from the abnormal hemisphere, and ictal single-photon emission computed tomography demonstrated ipsilateral regional hyperperfusion. Functional hemispherectomy was performed after electrocorticography revealed near-continuous spike and slow-wave discharges over the affected hemisphere. Histopathology revealed Taylor-type cortical dysplasia. Postoperatively, both patients are seizure free on antiepileptic drug monotherapy and have shown improved developmental status. Their favorable outcome demonstrates that children with early infantile epileptic encephalopathy due to unilateral malformations of cortical development may benefit from early surgical intervention.
