OBJECTIVE: Tuberous sclerosis complex (TSC) is a genetic disorder primarily characterized by the development of multisystem benign tumors. Epilepsy is the most common neurologic manifestation, affecting 80%-90% of TSC patients. The diffuse structural brain abnormalities and the multifocal nature of epilepsy in TSC pose diagnostic challenges when evaluating patients for epilepsy surgery.METHODS: We retrospectively reviewed the safety experience and efficacy outcomes of five adult TSC patients who were treated with direct brain-responsive neurostimulation (RNS System, NeuroPace, Inc).RESULTS: The average follow-up duration was 20months. All five patients were responders (=50% disabling seizure reduction) at last follow-up. The median reduction in disabling seizures was 58% at 1year and 88% at last follow-up. Three of the five patients experienced some period of seizure freedom ranging from 3months to over 1year.SIGNIFICANCE: In this small case series, we report the first safety experience and efficacy outcomes in patients with TSC-associated drug-resistant focal epilepsy treated with direct brain-responsive neurostimulation.
View details for DOI 10.1002/epi4.12481
View details for PubMedID 34033253