Resective epilepsy surgery in tuberous sclerosis complex: a nationwide multicentre retrospective study from China

Abstract

At least 50% of patients with tuberous sclerosis complex present with intractable epilepsy; for these patients, resective surgery is a treatment option. Here, we report a nationwide multicentre retrospective study and analyse the long-term seizure and neuropsychological outcomes of epilepsy surgery in patients with tuberous sclerosis complex. There were 364 patients who underwent epilepsy surgery in the study. Patients’ clinical data, postoperative seizure outcomes at 1-, 4-, and 10-year follow-ups, preoperative and postoperative intelligence quotients, and quality of life at 1-year follow-up were collected. The patients’ ages at surgery were 10.35 ± 7.70 years (range: 0.5–47). The percentage of postoperative seizure freedom was 71% (258/364) at 1-year, 60% (118/196) at 4-year, and 51% (36/71) at 10-year follow-up. Influence factors of postoperative seizure freedom were the total removal of epileptogenic tubers and the presence of outstanding tuber on MRI at 1- and 4-year follow-ups. Furthermore, monthly seizure (versus daily seizure) was also a positive influence factor for postoperative seizure freedom at 1-year follow-up. The presence of an outstanding tuber on MRI was the only factor influencing seizure freedom at 10-year follow-up. Postoperative quality of life and intelligence quotient improvements were found in 43% (112/262) and 28% (67/242) of patients, respectively. Influence factors of postoperative quality of life and intelligence quotient improvement were postoperative seizure freedom and preoperative low intelligence quotient. The percentage of seizure freedom in the tuberectomy group was significantly lower compared to the tuberectomy plus and lobectomy groups at 1- and 4-year follow-ups. In conclusion, this study, the largest nationwide multi-centre study on resective epilepsy surgery, resulted in improved seizure outcomes and quality of life and intelligence quotient improvements in patients with tuberous sclerosis complex. Seizure freedom was often achieved in patients with an outstanding tuber on MRI, total removal of epileptogenic tubers, and tuberectomy plus. Quality of life and intelligence quotient improvements were frequently observed in patients with postoperative seizure freedom and preoperative low intelligence quotient.

Downregulated GPR30 expression in the epileptogenic foci of female patients with focal cortical dysplasia type IIb and tuberous sclerosis complex is correlated with 18F‐FDG PET‐CT values

“…The epileptogenic tubers were defined according to our previous study (34). Some prominent non-epileptogenic tubers that were >3-4 cm, had a nidus of calcification, and located in the nonfunctional brain areas within the operative fields, were resected due to their potential to develop into epileptogenic tubers.…”

Surgery for drug‐resistant tuberous sclerosis complex‐associated epilepsy: who, when, and what

“…These alterations are not only visible at the tuber centre but also in the perituberal tissue, suggesting that the tissue surrounding the epileptogenic tuber is also epileptogenic. Indirect confirmation of this hypothesis is the large number of seizure‐free patients after tuberectomy plus corticectomy versus tuberectomy alone [49, 50].…”

“…only visible at the tuber centre but also in the perituberal tissue, suggesting that the tissue surrounding the epileptogenic tuber is also epileptogenic. Indirect confirmation of this hypothesis is the large number of seizure-free patients after tuberectomy plus corticectomy versus tuberectomy alone [49,50]. In addition to delineating the resection margins in preoperative plans, accurate definition of TSC-lesion MRI has important implications for clinicians with respect to correlation of the clinical severity to the extent of cortical abnormality.…”

Medical treatment of tuberous sclerosis-related epilepsy

“…Alongside drug therapy, the eligibility for surgical treatment in these patients should be considered early in the treatment course. In cases with a well-delimited zone of seizure onset, the long-term (10 years) Engel I results range between 47 and 51% [36,37]. Combining both treatment approaches in patient management may achieve maximal sustained seizure-control with better neuropsychological outcomes.…”