Epilepsy surgery in tuberous sclerosis: a systematic review

Abstract

Purpose: Tuberous sclerosis complex (TSC) is often associated with intractable epilepsy. Although epilepsy surgery has gained interest in recent years uncertainties exist about which patients are good surgical candidates. A systematic review of the available literature has been undertaken to assess the overall outcome of epilepsy surgery and identify risk factors of seizure recurrence.

Methods: We searched MEDLINE, Embase, and bibliographies of reviews and book chapters to identify articles published in English since 1960. Twenty-five articles, describing postoperative seizure outcome and type of surgery in 177 TSC patients, were included in this study. Seizure outcome was analyzed both as seizure freedom and good outcome, including patients with >90% seizure reduction.

Results: Seizure freedom was achieved in 101 patients (57%). Seizure frequency was improved by > 90% in 32 patients (18%). Moderate or severe intellectual disability (IQ < 70) (RR 1.8; 95% CI 1.2-2.8) and the presence of tonic seizures (RR 1.7; 95 % CI 1.2-2.4) were related to seizure recurrence.

Conclusions: A relation between multiple seizures types with early onset, multiple cortical tubers and multifocal epileptogenicity, and poor outcome is not supported by this systematic analysis. Although there is considerable variation among studies reviewed here, the literature suggests that resective surgery may offer benefit in a selected population of TSC patients with drug-resistant epilepsy.