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Published on: May 16, 2019
Overview of therapeutic options for epilepsy
M Kuchenbuch1, C Chiron2, M Milh3
1Département de Pédiatrie et Génétique, CHU de Nancy, France.
Insights
Early seizure control in Tuberous Sclerosis Complex (TSC) epilepsy is crucial for cognitive and behavioral outcomes. Innovative treatments like vigabatrin initiated early show promise in managing TSC-related seizures and improving developmental prognosis.
Area of Science:
- Neurology
- Pediatrics
- Epileptology
Background:
- Tuberous Sclerosis Complex (TSC) epilepsy presents with diverse seizure types, including infantile spasms and focal seizures, significantly impacting cognitive and behavioral development.
- Early and effective seizure management is paramount for improving long-term outcomes in children with TSC.
Purpose of the Study:
- To review current and emerging therapeutic strategies for managing epilepsy in Tuberous Sclerosis Complex (TSC).
- To highlight specific treatment considerations and advancements for TSC-related seizures.
Main Methods:
- Review of current therapeutic options for TSC epilepsy, including first-line treatments, adjunctive therapies, and surgical interventions.
- Discussion of novel approaches such as early-onset vigabatrin treatment and mTOR inhibitors.
Main Results:
- Vigabatrin is a recommended first-line treatment for infantile spasms and early focal seizures in TSC.
- Cannabidiol and everolimus are approved adjunctive therapies for refractory seizures, alongside ketogenic diets.
- Surgical options, including stereotactic laser thermocoagulation, are essential for drug-resistant epilepsy, with potential for sequential interventions on epileptogenic tubers.
- Early initiation of vigabatrin treatment demonstrates efficacy in preventing or mitigating epilepsy development in TSC.
Conclusions:
- A multi-faceted approach combining pharmacotherapy, dietary interventions, and surgery is essential for optimal seizure control in TSC.
- Emerging treatments and early intervention strategies offer improved prospects for managing TSC epilepsy and its associated developmental challenges.
Abstract:
Tuberous sclerosis (TSC) epilepsy includes infantile spasms and focal seizures before the age of 2 years, whereas focal seizures are predominant over 2 years and generalized seizures may occasionally be part of Lennox-Gastaut syndrome. The better and earlier the seizure control, the better the child's subsequent cognitive and behavioral prognosis. As for epilepsy of other causes, therapeutic options depend on the type of seizure/epilepsy, age and drug resistance, but there are significant specificities for TSC. (1) As first-line treatment, vigabatrin is unanimously recommended for infantile spasms and focal seizures before 2 years and is also widely used for seizures over 2 years, as are levetiracetam and carbamazepine. (2) If seizures persist (about 40% of children and adolescents), cannabidiol and everolimus, an inhibitor of the mTOR pathway, have recently been approved as adjunctive therapy to the arsenal of antiseizure medications authorized for this age group and to the ketogenic diet. (3) Surgery is an essential treatment option in cases of drug resistance and should be discussed as soon as two treatments have failed. Presurgical investigations and operating techniques have recently progressed spectacularly, for example laser thermocoagulation with stereotactic location. A particularity of TSC is the possibility of sequential interventions on several epileptogenic tubers. (4) Finally, the innovative principle of initiating "pre-seizure" treatment with vigabatrin from the first months of life has just proven effective on the subsequent development of epilepsy in TSC. © 2022 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.
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