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针对结节性硬化症所致难治性癫痫发作的检测与治疗进行额叶反应性神经刺激:病例说明

Responsive neurostimulation of the frontal lobe for the detection and treatment of seizures in intractable epilepsy due to tuberous sclerosis complex: illustrative case.

作者信息

Brabant Paige J, Beaudreault Cameron P, Wolf Steven M, McGoldrick Patricia E, Ghatan Saadi, Muh Carrie R

机构信息

New York Medical College, Valhalla, New York.

Department of Pediatric Neurology, Boston Children's Health Physicians, Hawthorne, New York.

出版信息

J Neurosurg Case Lessons. 2024 Oct 7;8(15). doi: 10.3171/CASE23411.

DOI:10.3171/CASE23411
PMID:39378523
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11465341/
Abstract

BACKGROUND

Responsive neurostimulation (RNS) is often considered to be a palliative therapy for drug-resistant epilepsy (DRE) and is generally not considered to be a treatment for patients with tuberous sclerosis complex (TSC). Here, the authors present the case of a 24-year-old male with TSC who obtained seizure freedom following RNS device implantation.

OBSERVATIONS

Prior to RNS device implantation, the patient underwent tuberectomies, subependymal giant cell astrocytoma resection, vagus nerve stimulator placement, and left frontal lobe resection but continued to have frequent seizures. An RNS device was implanted, which initially led to a decrease in seizures, but he continued to have 12 seizures per month. He then underwent lead revision for stimulation via a different electrode. After that lead change, he had no seizures for almost 3 years. In the following 3 years, he had two episodes of breakthrough seizures, both of which occurred with medication weans. Although the patient still requires antiseizure medication, he has had years of seizure freedom with RNS therapy.

LESSONS

This study exhibits the potential effectiveness of RNS therapy for patients with TSC and DRE. RNS should be considered for patients with TSC when other therapies have not sufficiently treated their epilepsy. https://thejns.org/doi/10.3171/CASE23411.

摘要

背景

反应性神经刺激(RNS)通常被认为是一种针对药物难治性癫痫(DRE)的姑息治疗方法,一般不被视为结节性硬化症(TSC)患者的治疗手段。在此,作者介绍了一例24岁患有TSC的男性患者,在植入RNS设备后实现了无癫痫发作。

观察结果

在植入RNS设备之前,该患者接受了结节切除术、室管膜下巨细胞星形细胞瘤切除术、迷走神经刺激器置入术和左额叶切除术,但仍频繁发作癫痫。植入了RNS设备,最初癫痫发作有所减少,但他每月仍有12次发作。随后他接受了通过不同电极进行刺激的电极修正术。在那次电极更换后,他近3年没有癫痫发作。在接下来的3年里,他有两次突破性癫痫发作,均在减药时发生。尽管该患者仍需要抗癫痫药物治疗,但通过RNS治疗他已多年无癫痫发作。

经验教训

本研究展示了RNS治疗对TSC和DRE患者的潜在有效性。当其他疗法未能充分治疗癫痫时,TSC患者应考虑采用RNS治疗。https://thejns.org/doi/10.3171/CASE23411 。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6ea0/11465341/86de4f2e7048/CASE23411_figure_2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6ea0/11465341/6e717f6d7107/CASE23411_figure_1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6ea0/11465341/86de4f2e7048/CASE23411_figure_2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6ea0/11465341/6e717f6d7107/CASE23411_figure_1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6ea0/11465341/86de4f2e7048/CASE23411_figure_2.jpg

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本文引用的文献

1
Responsive Neurostimulation for the Treatment of Children With Drug-Resistant Epilepsy in Tuberous Sclerosis Complex.响应性神经刺激治疗结节性硬化症复杂型耐药性癫痫患儿
Pediatr Neurol. 2023 Aug;145:97-101. doi: 10.1016/j.pediatrneurol.2023.05.008. Epub 2023 May 19.
2
Brain-Responsive Neurostimulation for the treatment of adults with epilepsy in tuberous sclerosis complex: A case series.脑反应性神经刺激治疗结节性硬化症成人癫痫:病例系列。
Epilepsia Open. 2021 Jun;6(2):419-424. doi: 10.1002/epi4.12481. Epub 2021 Mar 13.
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Economic burden associated with tuberous sclerosis complex in patients with epilepsy.
与癫痫患者结节性硬化症相关的经济负担。
Epilepsy Behav. 2020 Nov;112:107494. doi: 10.1016/j.yebeh.2020.107494. Epub 2020 Oct 5.
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Real-world experience with direct brain-responsive neurostimulation for focal onset seizures.直接针对大脑反应的神经刺激治疗局灶性发作性癫痫的真实世界经验。
Epilepsia. 2020 Aug;61(8):1749-1757. doi: 10.1111/epi.16593. Epub 2020 Jul 13.
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Epilepsy treatment patterns among patients with tuberous sclerosis complex.结节性硬化症患者的癫痫治疗模式。
J Neurol Sci. 2018 Aug 15;391:104-108. doi: 10.1016/j.jns.2018.06.011. Epub 2018 Jun 15.
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Two-year seizure reduction in adults with medically intractable partial onset epilepsy treated with responsive neurostimulation: final results of the RNS System Pivotal trial.反应性神经刺激治疗药物难治性部分性发作癫痫成人患者 2 年发作减少:RNS 系统关键试验的最终结果。
Epilepsia. 2014 Mar;55(3):432-41. doi: 10.1111/epi.12534. Epub 2014 Feb 22.
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Electrocorticographic evidence of perituberal cortex epileptogenicity in tuberous sclerosis complex.结节性硬化症中肾小管周围皮质致痫性的皮质脑电图证据。
J Neurosurg Pediatr. 2012 Nov;10(5):376-82. doi: 10.3171/2012.8.PEDS1285. Epub 2012 Sep 21.