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

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...

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Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8166788/
https://www.ncbi.nlm.nih.gov/pubmed/34033253
http://dx.doi.org/10.1002/epi4.12481
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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
Patra, Sanjay E.
Brown, Mesha‐Gay
Haneef, Zulfi
author_facet 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_sort McDermott, Danielle S.
collection PubMed
description 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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spelling pubmed-81667882021-06-05 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 Epilepsia Open Short Research Articles 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. John Wiley and Sons Inc. 2021-03-13 /pmc/articles/PMC8166788/ /pubmed/34033253 http://dx.doi.org/10.1002/epi4.12481 Text en © 2021 The Authors. Epilepsia Open published by Wiley Periodicals LLC on behalf of International League Against Epilepsy https://creativecommons.org/licenses/by/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Short Research Articles
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
Brain‐Responsive Neurostimulation for the treatment of adults with epilepsy in tuberous sclerosis complex: A case series
title Brain‐Responsive Neurostimulation for the treatment of adults with epilepsy in tuberous sclerosis complex: A case series
title_full Brain‐Responsive Neurostimulation for the treatment of adults with epilepsy in tuberous sclerosis complex: A case series
title_fullStr Brain‐Responsive Neurostimulation for the treatment of adults with epilepsy in tuberous sclerosis complex: A case series
title_full_unstemmed Brain‐Responsive Neurostimulation for the treatment of adults with epilepsy in tuberous sclerosis complex: A case series
title_short Brain‐Responsive Neurostimulation for the treatment of adults with epilepsy in tuberous sclerosis complex: A case series
title_sort brain‐responsive neurostimulation for the treatment of adults with epilepsy in tuberous sclerosis complex: a case series
topic Short Research Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8166788/
https://www.ncbi.nlm.nih.gov/pubmed/34033253
http://dx.doi.org/10.1002/epi4.12481
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