Brain-Responsive Neurostimulation for the treatment of adults with epilepsy in tuberous sclerosis complex: A case series

McDermott, Danielle S; Mirro, Emily A; Fetrow, Kirsten; Burdette, David E; Chen, Stephanie; Hopp, Jennifer; Masel, Todd; Johnson, Emily A; Elefant, Felicia M K; Le, Scheherazade; Patra, Sanjay E; Brown, Mesha-Gay; Haneef, Zulfi

Author

McDermott, Danielle S
Mirro, Emily A
Fetrow, Kirsten
Burdette, David E
Chen, Stephanie
Hopp, Jennifer
Masel, Todd
Johnson, Emily A
Elefant, Felicia M K
Le, Scheherazade

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Date

2021-02-27

Journal

Epilepsia Open

Publisher

Wiley-Blackwell

Type

Article

Metadata

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See at

https://doi.org/10.1002/epi4.12481

Abstract

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 20 months. All five patients were responders (≥50% disabling seizure reduction) at last follow-up. The median reduction in disabling seizures was 58% at 1 year and 88% at last follow-up. Three of the five patients experienced some period of seizure freedom ranging from 3 months to over 1 year. 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.

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