Object Tuberous sclerosis complex (TSC) is a multisystem autosomal dominant disorder resulting in hamartomas of several organs. Cortical tubers are the most prominent brain lesions in TSC. Treatment-resistant epilepsy often develops early in life in patients with TSC and is associated with severe intellectual and behavioral impairments. Seizures may remit following epilepsy surgery in selected cases, yet it remains unclear whether the tuber or the perituberal cortex is the source of seizure onset. In this study, the authors reviewed the onset of seizures in patients in whom depth electrodes had been placed within or adjacent to cortical tubers. Methods After obtaining institutional review board approval, the authors retrospectively reviewed data from 12 pediatric patients with multifocal TSC and treatment-resistant epilepsy who had undergone invasive intracranial electroencephalographic monitoring. Tubers were identified on postimplantation MRI, and all depth electrodes were located. Depth electrode contacts were classified visually as either tuber/perituberal cortex or nontuber/nonperituberal cortex. Board-certified clinical neurophysiologists reviewed the seizures to identify all electrodes involved in the ictal onset. Results Among 309 recorded seizures, 104 unique ictal onset patterns were identified. Of the 11 patients with electrodes recording in a tuber, 9 had seizure onsets involving the tuber. Similarly, of the 9 patients with perituberal recording electrodes, 7 had perituberal ictal onsets. Overall, there was no difference in the percentage of contacts involved in seizure onset between the tuber and perituberal cortex. In a subset of 7 patients in whom at least 1 depth electrode contact was within the tuber and 1 was in the perituberal cortex, there was no difference between the percentage of tuber and perituberal onsets. Conclusions Findings demonstrated heterogeneity in the ictal onset patterns as well as involvement of the tuber and perituberal cortex within and between patients. Although the data are limited by the restricted region(s) sampled with intracranial electrodes, they do suggest that cortical hyperexcitability in TSC may derive from the tuber or surrounding cortex.

Summary Purpose:  Intracranial monitoring (IM) is a key diagnostic procedure for select patients with treatment‐resistant epilepsy (TRE). Seizure focus resection may improve seizure control in both lesional and nonlesional TRE. IM itself is not considered to have therapeutic potential. We describe a cohort of patients with improved seizure control following IM without resective surgery. Methods:  Over 12.5 years, 161 children underwent 496 surgeries including intracranial monitoring. We retrospectively reviewed the patients’ charts, operative reports, and radiologic scans, under an institutional review board–approved protocol. Key Findings:  Seventeen patients underwent only IM, without additional resective surgery, and seven had a dramatic improvement in their epilepsy; six of the seven patients are seizure‐free (Engel class I), and one rarely has seizures (Engel class II). All seven patients had frequent seizures that led to IM: either daily (five patients) or 1–2 per week (two patients). The mean age (± standard deviation, SD) at seizure onset was 1.6 ± 1.3 years (range 0.5–4 years). Etiologies were tuberous sclerosis (3 patients), trauma (1 patient), and unknown (3 patients). Mean age at surgery (± SD) was 4.1 ± 2 years (range 1–7 years), and duration of epilepsy 2.5 ± 1.1 years (range 0.5–4 years). Duration of IM was 11.7 ± 5.6 days (5–19 days). Six patients had bilateral and one unilateral invasive electrodes. At last follow‐up, four patients required fewer antiepileptic drugs (AEDs), one had the same medication but a higher dose, and two patients were taking additional AEDs. Follow‐up was 30.6 ± 9.5 months (range 19–41 months). Significance:  Although uncommon, patients with TRE may improve after IM alone. The explanation for this observation remains unclear; however, perioperative medications including steroids, direct cortical manipulation, or other factors may influence the epileptogenic network.

No abstract