Background

Clinicians have questioned whether any disorder involving seizures and neural antibodies should be called “(auto)immune epilepsy.” The concept of “acute symptomatic seizures” may be more applicable in cases with antibodies against neural cell surface antigens. We aimed at determining the probability of achieving seizure-freedom, the use of anti-seizure medication (ASM), and immunotherapy in patients with either constellation. As a potential pathophysiological correlate, we analyzed antibody titer courses.

Methods

Retrospective cohort study of 39 patients with seizures and neural antibodies, follow-up ≥ 3 years.

Results

Patients had surface antibodies against the N-methyl-d-aspartate receptor (NMDAR, n = 6), leucine-rich glioma inactivated protein 1 (LGI1, n = 11), contactin-associated protein-2 (CASPR2, n = 8), or antibodies against the intracellular antigens glutamic acid decarboxylase 65 kDa (GAD65, n = 13) or Ma2 (n = 1). Patients with surface antibodies reached first seizure-freedom (88% vs. 7%, P < 0.001) and terminal seizure-freedom (80% vs. 7%, P < 0.001) more frequently. The time to first and terminal seizure-freedom and the time to freedom from ASM were shorter in the surface antibody group (Kaplan–Meier curves: P < 0.0001 for first seizure-freedom; P < 0.0001 for terminal seizure-freedom; P = 0.0042 for terminal ASM-freedom). Maximum ASM defined daily doses were higher in the groups with intracellular antibodies. Seizure-freedom was achieved after additional immunotherapy, not always accompanied by increased ASM doses. Titers of surface antibodies but not intracellular antibodies decreased over time.

Conclusion

Seizures with surface antibodies should mostly be considered acute symptomatic and transient and not indicative of epilepsy. This has consequences for ASM prescription and social restrictions. Antibody titers correlate with clinical courses.

中文翻译：

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与细胞表面抗原抗体相关的癫痫发作是急性症状，并不表示癫痫：长期数据的见解

背景

临床医生已经质疑是否将涉及癫痫发作和神经抗体的任何疾病称为“（自身）免疫性癫痫”。“急性症状性癫痫发作”的概念可能更适用于具有抗神经细胞表面抗原抗体的病例。我们旨在确定任一星座患者实现癫痫发作的可能性，使用抗癫痫药（ASM）以及进行免疫治疗的可能性。作为潜在的病理生理相关因素，我们分析了抗体效价过程。

方法

对39例癫痫和神经抗体患者进行回顾性队列研究，随访≥3年。

结果

患者有抵靠表面的抗体Ñ甲基d天冬氨酸受体（NMDAR，Ñ  = 6），富含亮氨酸的神经胶质瘤失活蛋白1（LGI1，Ñ  = 11），接触蛋白相关蛋白2（CASPR2，Ñ  = 8） ，或针对细胞内抗原65 kDa（GAD65，n  = 13）或Ma2（n  = 1）的抗体。患有表面抗体的患者首次发作无癫痫发作（88％vs. 7％，P  <0.001）和终末发作无癫痫发作（80％vs. 7％，P <0.001）。时间第一和终端发作自由和时间从ASM自由均表面抗体组在短（Kaplan-Meier曲线：P  <0.0001第一发作自由; P  <0.0001终端发作自由; P  = 0.0042用于终端ASM自由）。具有细胞内抗体的组中，最大ASM定义的日剂量更高。额外的免疫治疗后无癫痫发作，但并不总是伴随着ASM剂量的增加。随着时间的推移，表面抗体的滴度会下降，但细胞内抗体的滴度不会下降。

结论

带有表面抗体的癫痫发作大多数应被视为急性症状和短暂发作，并不表示癫痫发作。这会对ASM处方和社会限制产生影响。抗体滴度与临床过程相关。