Surgical treatment of epilepsy in tuberous sclerosis

Abstract

Surgical treatment of patients with TSC and intractable epilepsy is most effective when a single tuber or epileptogenic area can be identified as the source of seizures and resected. This may be possible even when other tubers or diffuse EEG abnormalities are present. In patients with unlocalizable epileptic abnormalities, palliation may be obtained by CC.

Epilepsy surgery in tuberous sclerosis: a review

“…In these first reports, the patients selected had focal electroencephalographic discharges with little ambiguity in localization. In a study of 18 patients by Guerreiro et al, 14 12 patients had focal or regional EEG abnormalities directing resection. Six of the patients, however, had multiple lesions or nonlocalizable epilepsy due to generalized EEG abnormalities.…”

Epilepsy surgery and tuberous sclerosis complex: special considerations

“…Over the 40 years since that report, the published literature has consistently found that traditional resective epilepsy surgery can be very successful in reducing the seizure burden in carefully selected patients with TSC who harbor a single tuber and associated epileptogenic focus. 3,5,8,18,[26][27][28]34,35,37,41,52,57,75 Patients not meeting this selection criterion for surgery were offered a palliative approach, such as corpus callosotomy. 18 A review of the TSC epilepsy surgery literature indicates that patients with a well-localized single tuber and corresponding epileptogenic zone are excellent surgical candidates, but children not fitting this profile are often excluded from operative treatment because a poor outcome was anticipated.…”

“…Therefore, early identification of potential surgical candidates is critical for seizure control and optimal development. However, the published literature primarily contains reports on older children, 3,5,8,18,[26][27][28]34,35,37,41,52,57,75 with very few younger patients included in these series. This raises the question of whether a potential benefit is not seen because surgery is delayed beyond a potentially important developmental window.…”

“…Furthermore, some have observed interictal epileptiform discharges within tubers during intraoperative recording, and the resection of tubers associated with epileptiform activity has led to diminished seizure frequency. 3,5,8,18,[26][27][28]34,35,37,41,52,57,75 However, little direct evidence in the literature supports a clear correlation between the seizure focus and the tuber. Moreover, analysis of several surgical descriptions in the literature reveals that cortex adjacent to tubers is often resected during "tuberectomy."…”

“…25,72 Based on the experience of several investigators over the last 20 years, the efficacy of surgery is well established for controlling seizures in patients with TSC with concordant and localized neuroimaging and electrophysiological data. 3,5,8,18,[26][27][28]34,35,37,41,42,52,57,75 Independent investigators at a variety of international comprehensive epilepsy centers have come to the conclusion that epilepsy surgery is an excellent treatment option for the appropriately selected child with TSC. 3,5,8,18,[26][27][28]34,35,37,41,52,57,75 The goal of epilepsy treatment in TSC, as in the treatment of other types of epilepsy, is the elimination of seizures as soon as possible to optimize cognitive development and improve behavior and quality of life.…”