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

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

doi:10.1002/epi4.12481

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

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

Neurology

Research output: Contribution to journal › Article › peer-review

12 Scopus citations

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.

Original language	English (US)
Pages (from-to)	419-424
Number of pages	6
Journal	Epilepsia Open
Volume	6
Issue number	2
DOIs	https://doi.org/10.1002/epi4.12481
State	Published - Jun 2021

Keywords

refractory epilepsy
responsive neurostimulation
tuberous sclerosis complex

ASJC Scopus subject areas

Neurology
Clinical Neurology

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10.1002/epi4.12481

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McDermott, D. S., Mirro, E. A., Fetrow, K., Burdette, D. E., Chen, S., Hopp, J., Masel, T., Johnson, E. A., Elefant, F. M. K., Le, S., Patra, S. E., Brown, M. G., & Haneef, Z. (2021). Brain-Responsive Neurostimulation for the treatment of adults with epilepsy in tuberous sclerosis complex: A case series. Epilepsia Open, 6(2), 419-424. https://doi.org/10.1002/epi4.12481

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

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.",

keywords = "refractory epilepsy, responsive neurostimulation, tuberous sclerosis complex",

author = "McDermott, {Danielle S.} and Mirro, {Emily A.} and Kirsten Fetrow and Burdette, {David E.} and Stephanie Chen and Jennifer Hopp and Todd Masel and Johnson, {Emily A.} and Elefant, {Felicia M.K.} and Scheherazade Le and Patra, {Sanjay E.} and Brown, {Mesha Gay} and Zulfi Haneef",

note = "Publisher Copyright: {\textcopyright} 2021 The Authors. Epilepsia Open published by Wiley Periodicals LLC on behalf of International League Against Epilepsy",

year = "2021",

month = jun,

doi = "10.1002/epi4.12481",

language = "English (US)",

volume = "6",

pages = "419--424",

journal = "Epilepsia Open",

issn = "2470-9239",

publisher = "Wiley-Blackwell Publishing Ltd",

number = "2",

}

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T1 - Brain-Responsive Neurostimulation for the treatment of adults with epilepsy in tuberous sclerosis complex

T2 - A case series

AU - McDermott, Danielle S.

AU - Mirro, Emily A.

AU - Fetrow, Kirsten

AU - Burdette, David E.

AU - Chen, Stephanie

AU - Hopp, Jennifer

AU - Masel, Todd

AU - Johnson, Emily A.

AU - Elefant, Felicia M.K.

AU - Le, Scheherazade

AU - Patra, Sanjay E.

AU - Brown, Mesha Gay

AU - Haneef, Zulfi

PY - 2021/6

Y1 - 2021/6

N2 - 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.

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

Abstract

Keywords

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