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Neurology-LGI1-抗体脑炎者,行免疫治疗仍会出现慢性癫痫

2021-07-17 Freeman MedSci原创

LGI1-抗体脑炎者,行免疫治疗仍会出现慢性癫痫

靶向神经表面抗原的自身抗体已被越来越多地确定为脑炎和癫痫发作的一个原因。富含亮氨酸的胶质瘤灭活(leucine-rich glioma-inactivated 1,LGI1)抗体与边缘性脑炎和急性症状性癫痫发作(包括面肌张力障碍性发作(FBDS))有关,后者通常对抗癫痫药物治疗(ASM)耐受。

然而,在长期随访中,有高达35%的病人复发,而且在急性脑炎缓解后,也有慢性癫痫的报道。最近有几篇文章指出,由于针对神经细胞表面抗原(如LGI1)的抗体,脑炎患者出现了慢性癫痫的情况。在一项研究中,免疫治疗延迟和脑电图上出现发作性癫痫样放电被确定为与持续发作或复发有关的风险因素。然而,考虑到这种情况的相对罕见性和已发表的系列文章中的样本量小,可能存在这些次优结果的其他风险因素。此外,慢性类固醇疏散免疫疗法在治疗这种疾病方面的疗效还没有得到很多关注。

藉此, 布朗大学的Kelsey M. Smith等人,确定LGI1-抗体脑炎患者的长期发作结果,并确定与复发和发展为慢性癫痫有关的临床因素。最后,评估慢性类固醇疏通免疫疗法在长期结果中的价值。

他们在梅奥诊所的电子病历和神经免疫学实验室记录中确定了与LGI1-抗体脑炎相关的癫痫发作患者,且从发病起随访时间≥24个月。审查病历以确定临床因素、发作类型、影像学、治疗、复发的发生和结果。进行逻辑回归分析以确定慢性癫痫发展的预测因素。采用单变量Cox比例危害回归法来研究基线特征对复发风险的影响。


他们发现了49名患有LGI1-抗体脑炎和急性症状性癫痫发作的患者。几乎所有患者(n=48,98%)都接受了免疫治疗。在最后一次随访中,有8人明确,2人可能有慢性癫痫(10/49,20.4%)。

女性(P=0.048)和发病时年龄较小(P=0.02)与慢性癫痫的发展有关。

20人(40.8%)出现复发,首次复发的中位时间为7.5个月(范围3-94个月)。慢性类固醇疏通免疫疗法的初始治疗与复发风险的降低有关(危险比=0.28,95%CI 0.11-0.73,P=0.009)。

这个研究的重要意义在于发现了,尽管进行了积极的免疫治疗,20.4%的LGI1-抗体脑炎患者发生了慢性癫痫。慢性癫痫的危险因素是女性和较早发病的年龄。在长期随访的患者中,40.8%的患者发生了复发,慢性类固醇疏通免疫疗法与较低的复发率有关

原文出处
Smith KM, Dubey D, Liebo GB, Flanagan EP, Britton JW. Clinical Course and Features of Seizures Associated With LGI1-Antibody Encephalitis. Neurology. 2021 Jul 7:10.1212/WNL.0000000000012465. doi: 10.1212/WNL.0000000000012465. Epub ahead of print. PMID: 34233939.

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    2022-01-02 yinhl1978
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    2021-07-18 Avigo

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