Surgical Treatment of Epileptic Encephalopathy with Spike-and-Wave Activation in Sleep Associated with...

Epileptic encephalopathy with spike-and-wave activation in sleep (EE-SWAS) is a rare childhood-onset epileptic encephalopathy, defined by a typical electrographic pattern with mostly continuous slow spike-wave complexes during non-REM sleep. It may be cryptogenic or secondary to genetic or structural etiology. It might be related to progressive and irreversible cognitive deterioration; under these circumstances, there is an urgency for fast adequate treatment. Polymicrogyria is a common brain malformation and epilepsy can occur in up to 90% of the patients. Extensive polymicrogyria can be a cause of EE-SWAS and treatment refractoriness. In these patients, surgical treatment might be considered.

We report on an 8-year-old child who presented with electrographic findings compatible with EE-SWAS and hemiparesis associated with right hemispheric polymicrogyria who successfully underwent hemispherectomy as primary treatment. Surgery is rarely considered for these patients.

This report highlights the importance of considering surgery as an early treatment modality in selected cases.