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新发难治性癫痫持续状态伴潜在自身免疫病因:一例报告

New-Onset Refractory Status Epilepticus with Underlying Autoimmune Etiology: a Case Report.

作者信息

Brunker Lucille, Hirst Priscilla, Schlesinger Joseph J

机构信息

1Department of Anesthesiology, Vanderbilt University Medical Center, Nashville, TN USA.

2Department of Medicine, Montefiore Health System, New Rochelle, United States.

出版信息

SN Compr Clin Med. 2020;2(1):103-107. doi: 10.1007/s42399-019-00185-z. Epub 2019 Nov 28.

DOI:10.1007/s42399-019-00185-z
PMID:32435752
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7223986/
Abstract

Management of new-onset refractory status epilepticus and the approach to burst suppression variable is often challenging. We present the unusual case of a previously healthy 18-year-old male with new-onset status epilepticus admitted to the neurologic intensive care unit for 70 days. Despite treatment with multiple anti-epileptic drugs in addition to IV anesthetics, burst suppression was initially unsustainable and the patient remained in super-refractory status epilepticus. Extensive evaluation revealed an underlying autoimmune-mediated etiology with positivity for glutamic acid decarboxylase-65 antibody. Clinical response with a goal of 1-2 bursts per screen on EEG monitor was eventually achieved after a course of rituximab and plasma exchange therapy as well as a 7-day barbiturate coma with a regimen of clobazam, lacosamide, Keppra, and oxcarbazepine followed by a slow taper of phenobarbital and the addition of fosphenytoin. Remarkably, the patient was subsequently discharged to a rehabilitation facility with complete neurologic recovery. We discuss treatment strategies for new-onset refractory status epilepticus and highlight the role of rapid initiation of burst suppression with high-dose IV anesthetics to ensure neuroprotection while the underlying etiology is addressed with immune-modulating therapy.

摘要

新发难治性癫痫持续状态的管理以及达到爆发抑制变量的方法通常具有挑战性。我们报告了一例不同寻常的病例,一名既往健康的18岁男性,新发癫痫持续状态,入住神经重症监护病房70天。尽管除静脉麻醉剂外还使用了多种抗癫痫药物进行治疗,但最初爆发抑制难以维持,患者仍处于超难治性癫痫持续状态。广泛评估发现潜在的自身免疫介导病因,谷氨酸脱羧酶-65抗体呈阳性。在接受利妥昔单抗和血浆置换治疗疗程以及7天巴比妥类药物昏迷,并采用氯巴占、拉科酰胺、左乙拉西坦和奥卡西平方案,随后缓慢减量苯巴比妥并加用磷苯妥英后,最终在脑电图监测下实现了以每屏1-2次爆发为目标的临床反应。值得注意的是,患者随后出院至康复机构,神经功能完全恢复。我们讨论了新发难治性癫痫持续状态的治疗策略,并强调了在使用免疫调节疗法解决潜在病因时,迅速启动高剂量静脉麻醉剂进行爆发抑制以确保神经保护的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/104c/7223986/0515ffd1b89f/42399_2019_185_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/104c/7223986/0515ffd1b89f/42399_2019_185_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/104c/7223986/0515ffd1b89f/42399_2019_185_Fig1_HTML.jpg

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Front Neurol. 2019 Feb 13;10:25. doi: 10.3389/fneur.2019.00025. eCollection 2019.
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Clinical Management of Epilepsy With Glutamic Acid Decarboxylase Antibody Positivity: The Interplay Between Immunotherapy and Anti-epileptic Drugs.谷氨酸脱羧酶抗体阳性癫痫的临床管理:免疫疗法与抗癫痫药物之间的相互作用
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