Chen Stephanie H, O'Dea Pamela K, Sianati Bahareh, Benavides David R
Department of Neurology, University of Maryland School of Medicine, Baltimore, MD, United States.
Department of Neurology, Indiana University School of Medicine, Indianapolis, IN, United States.
Front Neurol. 2022 Nov 2;13:1028290. doi: 10.3389/fneur.2022.1028290. eCollection 2022.
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.
自身免疫性脑炎（AE）在急性期常伴有癫痫发作。癫痫发作通常对抗癫痫药物（ASM）难治，但已证明对免疫调节疗法有反应。一部分AE患者在急性AE发作多年后仍持续患有难治性癫痫，最近被命名为“自身免疫相关性癫痫（AAE）”。AAE的最佳治疗方法尚未确定。此外，神经调节和免疫疗法在AAE中的疗效尚未得到充分证实。在此，我们报告一名可能自身抗体阴性的AE患者，他最初表现为新发难治性癫痫持续状态（NORSE）。在急性发作后，他继续频繁发作癫痫，对四种治疗剂量的ASM均难治。为了诊断和治疗目的植入了反应性神经刺激（RNS，NeuroPace）系统，但癫痫发作频率变化极小。尽管使用了ASM和神经刺激，癫痫发作仍频繁，因此他接受了由大剂量静脉注射（IV）皮质类固醇和静脉注射免疫球蛋白（IVIG）组成的免疫疗法试验。尽管在治疗方案中增加了免疫疗法，但患者的癫痫发作频率在临床或脑电图方面均无明显变化。该病例不支持使用免疫疗法治疗AAE，并说明了在AAE患者管理中需要达成共识指南。此外，使用皮质脑电图（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。
