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Developmental epileptic encephalopathy with spike-wave activation in sleep (DEE-SWAS) is an epilepsy syndrome of childhood characterized by developmental delay or regression with significant abnormal epileptiform activity during sleep. In some cases, DEE-SWAS with an identified focal lesion is treated with surgical resection. The authors report an unusual case of focal DEE-SWAS that was successfully treated via transorbital resection with intraoperative electrocorticography (iECoG). The patient is an 11-year-old boy with a history of medication-responsive seizures who presented with cognitive and language decline. Electroencephalography demonstrated abnormal electrographic activity during sleep consistent with DEE-SWAS. Imaging and electrographic studies identified a probable epileptogenic zone anterior and adjacent to Broca’s area. He underwent stereoelectroencephalography followed by focal resection with iECoG, which resulted in resolution of EEG abnormalities, improved word finding, and no further cognitive decline. While DEE-SWAS is not typically managed with surgical intervention, focal resection in carefully selected patients can achieve favorable outcomes.

Stereoelectroencephalography (SEEG) is an increasingly popular invasive monitoring approach to epilepsy surgery in patients with drug-resistant epilepsies. The technique allows a three-dimensional definition of the epileptogenic zones (EZ) in the brain. It has been shown to be safe and effective in adults and older children but has been used sparingly in children less than two years old due to concerns about pin fixation in thin bone, registration accuracy, and bolt security. As such, most current series of pediatric invasive EEG explorations do not include young participants, and, when they do, SEEG is often not utilized for these patients. Recent national survey data further suggests SEEG is infrequently utilized in very young patients. We present a novel case of SEEG used to localize the EZ in a 17-month-old patient with thin cranial bone, an open fontanelle, and severe drug-resistant epilepsy due to tuberous sclerosis complex (TSC), with excellent accuracy, surgical results, and seizure remission.