Epilepsy surgery in tuberous sclerosis complex (TSC): emerging techniques and redefinition of treatment goals
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ANNUAL ISSUE PAPER
Epilepsy surgery in tuberous sclerosis complex (TSC): emerging techniques and redefinition of treatment goals Jeffrey M. Treiber 1,2 & Daniel J. Curry 1,2 & Howard L. Weiner 1,2 & Jonathan Roth 3 Received: 30 April 2020 / Accepted: 28 May 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Epilepsy occurs in nearly all patients with tuberous sclerosis and is often refractory to medical treatment. The definition of surgical candidacy in these patients has broadened in recent years due to philosophical and technological advances. The goals of surgery have shifted to focusing on quality of life and maximizing neurodevelopmental potential in patients unable to obtain seizure freedom. Novel diagnostic, ablative, and neuromodulatory techniques have been developed that may help patients that were previously considered inoperable to have an improved quality of life. In the coming years, it is expected that these techniques will be further refined and lead to an improvement of neurological prognosis in patients with tuberous sclerosis. Keywords Tuberous sclerosis . Epilepsy . Surgery . Laser ablation . Stereo EEG
Epilepsy in TSC Epilepsy is a hallmark of TSC, occurring in about 80–90% of TSC patients. The origin of epileptic activity is associated with cortical tubers and is refractory to anti-epileptic drugs (AEDs) in about 50% of patients. Prolonged refractory epilepsy is associated with impaired neurocognition, while early seizure control is associated with improved neurocognitive outcomes and improvement of quality of life of both patient and families [1]. Typically, TSC-related epilepsy is multifocal, due to the multifocality of potentially epileptogenic tubers. These patients present with various associated seizures types, each with different semiology, frequency, and impact on the patient’s life. Despite the multifocality, TSC-related epilepsy is rarely generalized, and once a seizure focus and its associated epileptic network have been disrupted by surgery, the associated seizure disorder will often significantly improve. * Howard L. Weiner [email protected] 1
Department of Neurosurgery, Baylor College of Medicine, Houston, TX, USA
2
Division of Pediatric Neurosurgery, Department of Surgery, Texas Children’s Hospital, Houston, TX, USA
3
Department of Pediatric Neurosurgery, Dana Children’s Hospital, Tel Aviv Sourasky Medical Center, Tel-Aviv University, Tel-Aviv, Israel
Due to philosophical and technical advances, epilepsy surgery for TSC has experienced a tremendous growth in interest over the past 20 years in parallel with the expanding landscape of pediatric epilepsy surgery in general [2, 3]. Many more children are now considered potential candidates for surgery than previously, and both traditional and novel minimally invasive diagnostic and therapeutic approaches have been developed. Furthermore, the concept of treatment success has also shifted, as our understanding of the impact of refractory seizures on the developing brain has advanced. Surgery c
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