Abstract

Objective

Autoimmune limbic encephalitis (ALE) is characterized by memory impairment, psychiatric symptoms, and epileptic seizures. Though, the neuropsychological profile of ALE is not yet well defined. However, there is some evidence that neuropsychological impairments might exceed those related to the limbic system and that different autoantibodies (AABs) are associated with distinguishable pattern of neuropsychological impairments. We provide a comprehensive presentation of neuropsychological performance of ALE in an immune therapy-naïve sample.

Methods

We retrospectively analyzed 69 immunotherapy-naïve ALE-patients (26 seropositive—[8 LGI1-, 4 CASPR2-, 2 GABA_B-R-, 3 Hu-, 4 GAD65-, 2 Ma2-, 2 unknown antigen, and 1 Yo-AABs] and 43 seronegative patients, mean age 56.0 years [21.9–78.2], mean disease duration 88 weeks [0–572]). Neuropsychological evaluations comprised of the domains memory, attention, praxis, executive functions, language, social cognition, and psychological symptoms. We compared these functions between seronegative −, seropositive patients with AABs against intracellular neural antigens and seropositive patients with AABs against surface membrane neural antigens.

Results

No effect of AAB group on neuropsychological performance could be detected. Overall, ALE predominantly presents with deficits in long-term memory and memory recognition, autobiographical-episodic memory loss, impairment of emotion recognition, and depressed mood. Furthermore, deficits in praxis of pantomimes and imitations, visuo-construction, and flexibility may occur.

Conclusion

ALE shows a wide spectrum of neuropsychological impairments, which might exceed the limbic system, with no evidence of differences between AAB groups. Neuropsychological assessment for diagnosing ALE should include long-term memory, memory recognition, autobiographical-episodic memory, emotion recognition, and a detailed investigation of depression.

中文翻译：

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自身免疫性边缘脑炎的神经心理表现：来自免疫治疗初始队列的证据

摘要

客观的

自身免疫性边缘脑炎 (ALE) 的特征是记忆障碍、精神症状和癫痫发作。虽然，ALE 的神经心理学特征尚未明确定义。然而，有一些证据表明神经心理损伤可能超过与边缘系统相关的损伤，并且不同的自身抗体 (AAB) 与可区分的神经心理损伤模式相关。我们在未接受免疫治疗的样本中全面介绍了 ALE 的神经心理学表现。

方法

我们回顾性分析了 69 名未接受免疫治疗的 ALE 患者（26 名血清反应阳性——[8 名 LGI1-、4 名 CASPR2-、2 名 GABA _B -R- 、3 名 Hu-、4 名 GAD65-、2 名 Ma2-、2 名未知抗原和 1 名 Yo- AABs] 和 43 名血清反应阴性患者，平均年龄 56.0 岁 [21.9-78.2]，平均病程 88 周 [0-572]）。神经心理学评估包括记忆、注意力、实践、执行功能、语言、社会认知和心理症状等领域。我们比较了针对细胞内神经抗原的 AABs 血清阴性、血清阳性患者和针对表面膜神经抗原的 AABs 血清阳性患者之间的这些功能。

结果

未检测到 AAB 组对神经心理表现的影响。总体而言，ALE 主要表现为长期记忆和记忆识别缺陷、自传性情景记忆丧失、情绪识别障碍和情绪低落。此外，可能会出现哑剧和模仿、视觉建构和灵活性方面的缺陷。

结论

ALE 表现出广泛的神经心理障碍，可能超出边缘系统，没有证据表明 AAB 组之间存在差异。诊断 ALE 的神经心理学评估应包括长期记忆、记忆识别、自传情节记忆、情绪识别和对抑郁症的详细调查。