Role of responsive neurostimulation and immunotherapy in refractory epilepsy due to autoimmune encephalitis: A case report.
Role of responsive neurostimulation and immunotherapy in refractory epilepsy due to autoimmune encephalitis: A case report.
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DOI：
10.3389/fneur.2022.1028290
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发表时间：
2022
影响因子：
3.4
通讯作者：
中科院分区：
文献类型：
--
作者：
自身免疫性脑炎（AE）经常在急性发作时出现癫痫发作。癫痫发作通常对抗癫痫发作药物（ASM）无效，但已显示对免疫调节治疗有反应。AE患者的一个子集在急性AE表现后数年内继续患有难治性癫痫，最近被命名为“自身免疫相关性癫痫（AAE）”。AAE的最佳治疗尚未确定。此外，神经调节和免疫治疗在AAE中的疗效尚未得到充分证实。在这里，我们报告了一个可能的自身抗体阴性AE的患者，最初表现为新发难治性癫痫持续状态（NORSE）。在他的急性表现后，他继续频繁发作，在治疗剂量下对四种ASM无效。出于诊断和治疗目的，植入了反应性神经刺激（RNS®，NeuroPace）系统，癫痫发作频率变化极小。由于持续频繁癫痫发作，尽管ASM和神经刺激，他经历了免疫治疗试验，包括高剂量静脉注射（IV）皮质类固醇和静脉注射免疫球蛋白（IVIG）。尽管在其治疗方案中添加了免疫疗法，但患者的癫痫发作频率未发生显著的临床或电图变化。该病例不支持使用免疫疗法治疗AAE，并说明了在AAE患者管理中需要共识指南。此外，使用皮质电图（ECoG）数据提供了癫痫发作频率的客观替代指标;这可能支持早期神经调节在AAE管理中的作用。
Autoimmune encephalitis (AE) frequently presents with seizures in the acute setting. Seizures are often refractory to anti-seizure medications (ASM) but have been shown to be responsive to immunomodulatory therapies. A subset of patients with AE continues to have refractory epilepsy, recently named “autoimmune-associated epilepsy (AAE),” for years after the acute AE presentation. Optimal treatment for AAE has not been determined. Furthermore, the efficacy of neuromodulation and immunotherapy has not been well established in AAE. Here, we report a patient with probable autoantibody negative AE who initially presented with new onset refractory status epilepticus (NORSE). After his acute presentation, he continued to have frequent seizures that were refractory to four ASMs at therapeutic doses. A responsive neurostimulation (RNS®, NeuroPace) system was implanted for diagnostic and therapeutic purposes, with minimal change in seizure frequency. Due to continued frequent seizures despite ASMs and neurostimulation, he underwent a trial of immunotherapy consisting of high-dose intravenous (IV) corticosteroids and intravenous immunoglobulin (IVIG). Despite the addition of immunotherapy to his treatment regimen, the patient experienced no significant clinical or electrographic change in seizure frequency. This case does not support the use of immunotherapy for treatment of AAE and illustrates the need for consensus guidelines in the management of patients with AAE. Further, the use of electrocorticography (ECoG) data provided an objective surrogate measure of seizure frequency; this may support the role for early neuromodulation in the management of AAE.
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作者：
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DOI：
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发表时间：
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期刊：
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