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[Neurosurgical aspects in the treatment of children with tuberous sclerosis complex]
Jonathan Roth1, Itzhak Fried2, Shlomi Constantini2
1Department of Neurosurgery, Tel-Aviv Medical Center. jonaroth@gmail.com
Insights
Tuberous sclerosis complex (TSC) patients with refractory seizures can achieve high seizure control rates through epilepsy surgery. Neurosurgical interventions also address unique brain tumors in TSC, improving patient outcomes.
Area of Science:
- Neurosurgery
- Genetics
- Neurology
Background:
- Tuberous sclerosis complex (TSC) is a genetic disorder causing seizures, cognitive decline, and brain tumors.
- Refractory epilepsy and benign tumors (SEGAs) are significant challenges in TSC management.
- Historically, epilepsy surgery was rarely an option for TSC patients with refractory seizures.
Purpose of the Study:
- To review the neurosurgical aspects of treating Tuberous Sclerosis Complex.
- To highlight advancements in epilepsy surgery for TSC patients.
- To discuss management strategies for TSC-associated brain tumors.
Main Methods:
- Review of pre-surgical and surgical techniques for epilepsy in TSC.
- Analysis of outcomes for epilepsy surgery in TSC patients.
- Evaluation of treatment options for SEGAs in TSC.
Main Results:
- Epilepsy surgery offers a high rate of seizure control (around 75% seizure freedom) in TSC patients.
- Advanced techniques enable successful surgical intervention for refractory seizures.
- mTOR inhibitors and surgical approaches are key treatments for SEGAs, with combination therapy potentially reducing morbidity.
Conclusions:
- Neurosurgical interventions are increasingly effective for managing refractory epilepsy in Tuberous Sclerosis Complex.
- Comprehensive management of TSC involves addressing both seizures and unique brain tumors.
- Advancements in surgical and medical treatments offer improved outcomes for TSC patients.
Abstract:
Tuberous sclerosis complex (TSC) is a genetic syndrome, characterized by seizures, mental retardation, and a typical facial rash. Many patients have early onset seizures (usually during infancy) which are refractory to medical treatment. It has been shown that the neurocognitive decline in TSC patients is associated with the severity of seizures. The cerebral pathologies in TSC include - tubers (associated with seizures), benign tumors (subependymal giant cell astrocytomas - SEGAs), and intraventricular subependymal nodules (SENs). Until recent years, TSC patients with refractory seizures were only rarely considered to be suitable for epilepsy surgery. Over the past few years, several pre-surgical and surgical techniques have enabled patients to undergo epilepsy surgery with a high rate of seizure control. These techniques include invasive monitoring, mapping and resection of epileptogenic foci. The overall seizure outcome is around 75% seizure freedom. Additionally, benign brain tumors unique for TSC (SEGAs) present a treatment challenge. These tumors are typically located adjacent to the foramen of Monro. Without treatment the tumors may obstruct the CSF pathways leading to hydrocephalus. Current treatment options include surgical approaches, as well as medical treatment with mTOR inhibitors. The combination of medical and surgical treatments may decrease morbidity especially in partially resected tumors, multifocal tumors, or as neoadjuvant treatment in selected cases. In the current paper we review the neurosurgical aspects in the treatment of patients with TSC.
