Epilepsy Surgery in Tuberous Sclerosis: Is It the Holy Grail?

Introduction: Tuberous sclerosis (TS) is a chronic disorder with epilepsy starting during infancy or early childhood. Epilepsy in nearly 60% of patients, becomes pharmaco-resistant. Surgery results in good seizure outcome in 65 to 75% of selected patients. We describe 3 patients of TS, in whom a detailed presurgical work up revealed a well-defined focus, and who underwent intraoperative ultrasound (IOUS) guided electrocorticographic (ECoG) resection of tubers, resulting in seizure freedom.

Methods: Consecutive patients of TS with medically refractory epilepsy, presenting to Apollo Hospitals, Seshadripuram, Bangalore, from May 2018 to January 2023, were included. All patients underwent presurgical evaluation including, video EEG, MRI brain, FDGPET brain, and arterial spin labeling. Subsequently, the patients underwent IOUS-guided ECOG, and lesionectomies.

Results: Three patients were included. The mean age was 6.5 years. 2 were male. Presurgically, 2 tubers were identified as possible foci, using video EEG, FDG PET (hypometabolism), and ASL (decreased perfusion in 2 patients) in each of the patients. Intraoperatively, all patients underwent IOUS, which showed hyperechoic focus throughout the tuber, thus identifying the borders. ECoG was performed using strip electrodes on the surface and depth electrodes, introduced under IOUS guidance, into the tuber. Spiking was seen more frequently from the depth electrodes, guiding the lesionectomy. All patients achieved complete seizure freedom (Engel 1a) with a mean follow-up of 30 months. Histopathology revealed tubers in all patients.

Conclusions: Careful pre surgical work up, followed by IOUS-guided intraoperative ECOG, and resection, may help in achieving seizure freedom in TS.

Publication History

Article published online:
12 September 2024