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Optimal management of seizures associated with tuberous sclerosis complex: current and emerging options
1Department of Neurosurgery, University of Toronto, Toronto, ON, Canada.
Insights
Seizures affect most tuberous sclerosis complex patients, impacting development. Early diagnosis and varied treatments, including medication, surgery, and diet, are crucial for managing this condition.
Area of Science:
- Neurology
- Pediatrics
- Genetics
Background:
- Seizures are a common and debilitating manifestation in tuberous sclerosis complex (TSC), affecting 79%-90% of patients.
- Infantile spasms, often appearing in the first year of life, significantly impede neuropsychiatric, social, and cognitive development.
- The substantial individual and societal impact necessitates prompt identification and effective management of seizures in TSC.
Purpose of the Study:
- To review current and emerging therapeutic strategies for managing seizures in patients with tuberous sclerosis complex.
- To highlight the importance of early intervention in mitigating developmental consequences.
- To provide an overview of medical, surgical, and dietary approaches for seizure control in TSC.
Main Methods:
- Review of established and novel pharmacologic treatments, including vigabatrin, adrenocorticotropic hormone, and mTOR inhibitors.
- Analysis of surgical options, encompassing resective surgery for curative potential and palliative procedures like corpus callosotomy and vagus nerve stimulation.
- Evaluation of the ketogenic diet as an adjunctive or standalone therapy for refractory seizures.
Main Results:
- Medical management, including vigabatrin and ACTH, remains first-line therapy.
- mTOR inhibitors show promise for both seizures and subependymal giant cell astrocytoma.
- Resective surgery offers a potential cure in 56% of eligible patients, while palliative surgeries and the ketogenic diet address medically refractory cases.
Conclusions:
- A multi-modal approach integrating medical, surgical, and dietary interventions is essential for comprehensive seizure management in TSC.
- Early and aggressive treatment is critical to optimize neurodevelopmental outcomes.
- Ongoing research into novel therapies like mTOR inhibitors offers new hope for improved seizure control and associated complications in TSC.
Abstract:
Seizures are clinically significant manifestations associated with 79%-90% of patients with tuberous sclerosis complex. Often occurring within the first year of life in the form of infantile spasms, seizures interfere with neuropsychiatric, social, and cognitive development and carry significant individual and societal consequences. Prompt identification and treatment of seizures is an important focus in the overall management of tuberous sclerosis complex patients. Medical management, either after seizure onset or prophylactically in infants with electroencephalographic abnormalities, is considered first-line therapy. Vigabatrin and adrenocorticotropic hormone have emerged over the past few decades as mainstay pharmacologic modalities. Furthermore, emerging research on mammalian target of rapamycin inhibitors demonstrated promise for the management of seizures and subependymal giant cell astrocytoma. For appropriate surgical candidates with an epileptogenic zone associated with one or more glioneuronal hamartomas, ideally in noneloquent cortex, resective surgery can be considered, which provides a cure in 56% of patients. For medically refractory patients who do not meet criteria for curative surgery, palliative surgical approaches focused on reducing seizure burden, in the form of corpus callosotomy and vagus nerve stimulation, are alternative management options. Lastly, the ketogenic diet, a reemerging therapy based on the anticonvulsant effects of ketone bodies, can be utilized independently or in conjunction with other treatment modalities for the management of difficult-to-treat seizures.
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