Epilepsy surgery in tuberous sclerosis complex: Utilisation, satisfaction, and outcomes

Background: Tuberous sclerosis (TSC) is a rare genetic disorder associated with a high prevalence of drug-resistant epilepsy (DRE), for which epilepsy surgery may be a viable therapeutic option. National prospective studies with long-term follow-up (LTFU) and the identification of outcome predictors are needed.
Methods: Individuals with TSC but no history of previous epilepsy surgery were identified through a search of medical records at a Lund university hospital. The prospective Swedish national epilepsy-surgery register (SNESUR) was utilised to identify the surgical cohort. Questionnaires were used to obtain data regarding neuropsychiatric disorders and surgical satisfaction. Trends in symptoms and outcomes were compared before and after the year 2010.
Results: A search of medical records identified 52 patients: 43 (83%) had epilepsy, 27 (63%) had DRE at some point, and 20 (74%) were considered for surgery, 14 (52%) at a multidisciplinary epilepsy surgery round. At the 2-year follow-up after resection, seizure freedom increased from 1/9 (11%) in 1997-2010 to 5/9 (56%) in 2011-2018. Eight out of 15 patients were seizure-free at LTFU (3–15 years). In 9/15 cases (6/8 seizure-free), satisfaction was very high, and surgery was perceived as beneficial; 13/15 recommended surgery to others. Although the seizure-free group exhibited fewer neuropsychiatric problems, no significant differences were observed between the surgery and reference groups with a similar phenotype, but without surgery.
At the LTFU after callosotomy, 4/7 patients were free from drop attacks. None was highly satisfied, 2/5 patients perceived callosotomy as beneficial, and 1/5 as harmful.
Conclusion: DRE is highly prevalent in TSC, and two-fourths are considered for the only known cure of epilepsy; surgery. The rate of seizure freedom in Sweden has increased over time and is now comparable to some specialized TSC centres. Future studies are needed to identify predictors of outcomes and the most suitable patients for surgery.