Morano Alessandra, Cerulli Irelli Emanuele, Fortunato Francesco, Casciato Sara, Panzini Chiara, Milano Chiara, Versace Salvatore, Orlando Biagio, Iorio Raffaele, Tinelli Emanuele, Ruffolo Gabriele, Pizzanelli Chiara, Vogrig Alberto, Quarato Pierpaolo, Giallonardo Anna Teresa, Di Gennaro Giancarlo, Gambardella Antonio, Di Bonaventura Carlo
Department of Human Neurosciences, 'Sapienza' University of Rome, 00185 Rome, Italy.
Institute of Neurology, Department of Medical and Surgical Sciences, University 'Magna Graecia', 88100 Catanzaro, Italy.
J Neurol Sci. 2024 Dec 15;467:123288. doi: 10.1016/j.jns.2024.123288. Epub 2024 Nov 4.
Diagnosing autoimmune limbic encephalitis (ALE) in adults with new-onset seizures can be challenging, especially when seizures represent the predominant manifestation and MRI findings are not straightforward. By comparison with mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS), this study aimed to identify ictal electro-clinical features that might help clinicians recognize ALE-related seizures.
This retrospective, multi-centre study analysed the ictal semiology and EEG correlate of 116 video-EEG-captured seizures in 40 ALE patients and 45 ones recorded in 21 MTLE-HS subjects. The proportion of patients presenting each clinical feature on at least one occasion was compared between the study groups. Latent class analysis (LCA) was also performed.
Ictal features were overall more numerous in ALE than in MTLE-HS (33 vs 22), and LCA confirmed the intrinsic variability of ALE-related seizures. Hyperventilation served as a trigger only in ALE (4/40). Awareness impairment (p = 0.032), limb dystonic posturing (p = 0.009) and manual automatisms (p < 0.001) were significantly less common in ALE cases. Conversely, piloerection was observed only in ALE subjects, although it did not reach statistical significance (p = 0.289), as was the case for déjà-vu (p = 0.084), and sensory symptoms (p = 0.079). Regarding EEG, the type of ictal pattern differed significantly (p = 0.007).
This study shows that, despite the wide overlap with MTLE-HS, some ictal electro-clinical features could help clinicians suspect the autoimmune origin of adult-onset seizures. Moreover, autoimmune limbic seizures apparently shared similarities with 'temporal plus' epilepsy, which could partly account for the poor surgical outcomes and provide an interesting conceptual framework for future research.
诊断成年新发癫痫患者的自身免疫性边缘叶脑炎(ALE)具有挑战性,尤其是当癫痫为主要表现且MRI结果不明确时。通过与伴海马硬化的内侧颞叶癫痫(MTLE-HS)相比较,本研究旨在确定可能有助于临床医生识别ALE相关癫痫发作的发作期电临床特征。
这项回顾性多中心研究分析了40例ALE患者116次视频脑电图记录的癫痫发作的发作期症状学及脑电图相关性,以及21例MTLE-HS患者记录的45次癫痫发作情况。比较了研究组中至少有一次出现每种临床特征的患者比例。还进行了潜在类别分析(LCA)。
ALE发作期特征总体上比MTLE-HS更多(33个对22个),LCA证实了ALE相关癫痫发作的内在变异性。过度换气仅在ALE中作为触发因素(4/40)。意识障碍(p = 0.032)、肢体张力障碍姿势(p = 0.009)和手部自动症(p < 0.001)在ALE病例中明显较少见。相反,竖毛现象仅在ALE患者中观察到,尽管未达到统计学意义(p = 0.289),似曾相识感(p = 0.084)和感觉症状(p = 0.079)也是如此。关于脑电图,发作期模式类型有显著差异(p = 0.007)。
本研究表明,尽管与MTLE-HS有广泛重叠,但一些发作期电临床特征可帮助临床医生怀疑成人发作性癫痫的自身免疫起源。此外,自身免疫性边缘叶癫痫发作显然与“颞叶加”癫痫有相似之处,这可能部分解释了手术效果不佳的原因,并为未来研究提供了一个有趣的概念框架。