Epilepsy surgery and tuberous sclerosis complex: special considerations

Abstract

<jats:p>Epilepsy surgery for medically refractory seizures among patients with tuberous sclerosis complex (TSC) is a well-accepted treatment option. Many epilepsy centers around the world have published their experience over the past several years, supporting the idea that the best seizure control is obtained when a single tuber and associated epileptogenic zone is documented and targeted surgically. Recent advances in imaging and physiological techniques that reveal the epileptogenic zone have been used successfully in children with TSC who are being evaluated for surgery. As a result, a number of different surgical strategies have emerged, each reflecting the experience, strengths, and referral biases of the individual treating teams. Experience suggests that some patients with TSC who present with seizures that are difficult to localize and do not meet the classic selection criteria for epilepsy surgery may, nevertheless, benefit from sugery. Tuberectomy alone is often not sufficient for obtaining seizure control. Intracranial electrode recordings performed in a large number of children with TSC undergoing epilepsy surgery have raised new questions about the relationship of the cortical tuber to the epileptogenic zone in TSC. A careful assessment of the risks and benefits of any surgical strategy, compared with those associated with continued refractory epilepsy, should be considered by the treating team in conjunction with the patient's family. Epilepsy surgery has not only benefited many children with TSC, but it also facilitates the understanding of epileptogenesis in TSC.</jats:p>

Cytoarchitectural alterations are widespread in cerebral cortex in tuberous sclerosis complex

“…There is good evidence to suggest that resection of tubers alone may not be adequate for seizure control and that removal of surrounding tissue may play an important role in ultimate outcome. For example, in a recent series examining post-operative outcome in pediatric TSC patients, 10% of children underwent reoperation for recurrent seizures within the first 18 months after initial resection [4]. Interestingly, these patients required further Fig.…”

Long-term outcomes of resective epilepsy surgery after invasive presurgical evaluation in children with tuberous sclerosis complex and bilateral multiple lesions

“…This pessimistic outlook was challenged by the group at New York University, who pioneered a multistaged surgical approach based on detection of remaining adjacent epileptogenic tissue or distant secondary epileptogenic foci by another session of invasive monitoring, after a primary resection. 3,15 In a cohort of 25 children with TSC described by Weiner et al, 22 patients had 3-stage surgery, including 20 patients with resection of 2 or more tubers and 2 with bilateral resections. 14 The median durations of invasive monitoring sessions were 7 and 6 days, respectively.…”

“…Other patients have either been denied surgery or offered only palliative procedures because a poor outcome was anticipated. 3 There is growing concern about this intrinsic bias, and current epilepsy surgery in TSC is moving toward more extensive resections driven by concordance of imaging, neurophysiological, and other data. To substantiate the evidence in favor of such a data-driven approach, we present a single-center experience with resective surgery in TSC patients with medically refractory epilepsy who harbored multiple bilateral brain lesions.…”

Epilepsy Related to Developmental Tumors and Malformations of Cortical Development

“…Cortical tubers are considered to represent the neuropathologic substrate of epilepsy in TSC patients. Despite recent advances in imaging and electrophysiologic techniques, the functional role of tubers as the epileptogenic zone remains a matter of some debate [216][217][218][219]. Increasing evidence supports the importance of the perituberal cortex in TSC (for reviews see [165,218] and [219]).…”