Authors :
Presenting Author: Birce Taskin, MD – Baylor College of Medicine/Texas Children's Hospital
Daniel Curry, MD – Baylor College of Medicine/Texas Children's Hospital
Howard Weiner, MD – Baylor College of Medicine/Texas Children's Hospital
Vigi Katlowitz, MD – Baylor College of Medicine/Texas Children's Hospital
Michael Quach, MD – Baylor College of Medicine/Texas Children's Hospital
Deepankar Mohanty, MD – Baylor College of Medicine/Texas Children's Hospital
James Riviello, MD – Baylor College of Medicine/Texas Children's Hospital
Anne Anderson, MD – Baylor College of Medicine/Texas Children's Hospital
Eli Mizrahi, MD – Baylor College of Medicine/Texas Children's Hospital
Elaine Seto, MD – Baylor College of Medicine/Texas Children's Hospital
Irfan Ali, MD – Baylor College of Medicine/Texas Children's Hospital
Akshat Katyayan, MD – Baylor College of Medicine/Texas Children's Hospital
Kimberly Houck, MD – Baylor College of Medicine/Texas Children's Hospital
Cristina Trandafir, MD – Baylor College of Medicine/Texas Children's Hospital
Steven Lee, MD, PhD – Baylor College of Medicine/Texas Children's Hospital
Anuranjita Nayak, MD – Baylor College of Medicine/Texas Children's Hospital
Danielle Takacs, MD – Baylor College of Medicine/Texas Children's Hospital
Krystal Sully, MD – Baylor College of Medicine/Texas Children's Hospital
Sonali Sen, MD – Baylor College of Medicine/Texas Children's Hospital
Maria El-Hallal, MD – Baylor College of Medicine/Texas Children's Hospital
Brittnie Bartlett-Lee, MD – Baylor College of Medicine/Texas Children's Hospital
Ajay Thomas, MD, PhD – Baylor College of Medicine/Texas Children's Hospital
Daniel Davila-Williams, MD – Baylor College of Medicine/Texas Children's Hospital
Rohini Coorg, MD – Baylor College of Medicine/Texas Children's Hospital
Rationale:
Tuberous Sclerosis Complex (TSC) is a multisystem, autosomal dominant, neurocutaneous syndrome with an incidence of 1 in 6,000-22,000 live births. Most common neurological manifestation is epilepsy (80-90%) with most have drug-resistant epilepsy. Epilepsy surgery results in seizure freedom in >50% and should be considered for children with drug-resistant epilepsy. Epilepsy surgery planning in TSC has challenges due to multiple tubers. A multimodal approach is needed to distinguish between epileptogenic and non-epileptogenic tubers. However, little information is available in the literature describing which modality is the most concordant with the seizure onset zone in TSC patients.
Methods:
With this retrospective cohort study, we obtained data from patients with TSC that underwent epilepsy surgery from 2017 to 2022 at Texas Children’s Hospital. Study population inclusion criteria were ages < 21 years during the surgical procedure, drug refractory focal epilepsy with diagnosis of TSC, patients who underwent stereotactic electroencephalography (SEEG) placement or direct resection. Exclusion criteria was patients with unavailable clinical information. Demographic, seizure, presurgical work-up and surgical data was collected. The primary mode of data analysis was descriptive statistics.
Results:
We included 70 Tuberous Sclerosis patients who underwent epilepsy surgery at Texas Children’s Hospital in between 2017-2022. In 70 patients (35 female, 35 male), age range at seizure onset was 2 days to 5 years (median of 4 months). Focal impaired consciousness seizure (FICS) was the most frequent seizure type. Age range at the time of surgery was 11 months to 20 years (median age of 4 years). Sixty-eight (97%) patients underwent SEEG placement, only 2 patients (3%) underwent direct resection without SEEG. Final surgical procedures were laser interstitial thermal therapy (LITT) in 56 patients (80%), LITT plus resection in 4 patients (6%), LITT plus responsive neurostimulation (RNS) in 2 patients (3%), resection in 7 patients (10%) and deep brain stimulation (DBS) in 1 patient (1%). Seizure outcomes were analyzed at 1 year follow-up using Modified Engel Scale. Eight patients were excluded due to the lack of follow-up information. In 62 patients, 35 patients (56.5%) had good outcome with Engel Class 1-2 scoring, 15 patients (24.2%) were scored as Class 3 with some reduction of the seizures, 12 patients (19.3%) were scored as Class 4 with no worthwhile improvement. In the patients who have good surgical outcome, the most concordant modality is found to be Magnetoencephalography (MEG) comparing with the seizure onset zone on SEEG or surgical resection site (Figure 1).
Conclusions:
Our preliminary data showed most patients in our cohort underwent LITT alone or in combination with surgical options than resection or DBS. More than 50% of the patients had good outcome at 1 year follow up. In this group, MEG is found to be the most concordant pre-surgical modality and should be performed to distinguish between epileptogenic and non-epileptogenic tubers and to identify the epileptogenic zone.
Funding: N/A