Witt Juri-Alexander, Helmstaedter Christoph
Department of Epileptology, University Hospital Bonn (UKB), 53127 Bonn, Germany.
Brain Sci. 2021 Apr 29;11(5):576. doi: 10.3390/brainsci11050576.
Limbic encephalitis (LE) can cause dynamic and permanent impairment of cognition and behavior. In clinical practice, the question arises as to which cognitive and behavioral domains are affected by LE and which assessment is suited to monitor the disease progress and the success of treatment. Current findings on cognition and behavior in LE are reviewed and discussed based on current guidelines and consensus papers. In addition, we outline approaches for the neuropsychological monitoring of LE and its treatment. Dependent on disease acuity and severity, LE leads to episodic long-term memory dysfunction in different variants (e.g., anterograde memory impairment, accelerated long-term forgetting, and affection of autobiographical memory) and executive deficits. In addition, affective disorders are very common. More severe psychiatric symptoms may occur as well. In the course of the disease, dynamic phases with functional recovery must be differentiated from residual defect states. Evidence-based neuropsychological diagnostics should be conducted ideally before treatment initiation and reassessments are indicated when any progress is suggested, and when decisive anti-seizure or immunomodulatory treatment changes are made. Cognition and behavior may but must not run in synchrony with seizures, MRI pathology, or immune parameters. Cognitive and behavioral problems are integral aspects of LE and represent important biomarkers of disease acuity, progress, and therapy response beyond and in addition to parameters of immunology, neurological symptoms, and brain imaging. Thus, evidence-based neuropsychological assessments are essential for the diagnostic workup of patients with suspected or diagnosed limbic encephalitis, for treatment decisions, and disease and treatment monitoring.
边缘叶脑炎（LE）可导致认知和行为的动态性及永久性损害。在临床实践中，出现了这样的问题：LE会影响哪些认知和行为领域，哪种评估方法适合监测疾病进展及治疗效果。本文基于当前指南和共识文件，对LE患者认知和行为方面的现有研究结果进行综述和讨论。此外，我们概述了对LE及其治疗进行神经心理学监测的方法。根据疾病的严重程度和急缓，LE会导致不同类型的发作性长期记忆功能障碍（如顺行性记忆损害、加速性长期遗忘和自传体记忆受损）以及执行功能缺陷。此外，情感障碍也很常见。还可能出现更严重的精神症状。在疾病过程中，必须区分功能恢复的动态阶段和残留缺陷状态。理想情况下，应在开始治疗前进行基于证据的神经心理学诊断，当提示有任何进展、进行决定性的抗癫痫或免疫调节治疗改变时，需再次评估。认知和行为可能但不一定与癫痫发作、MRI病理或免疫参数同步。认知和行为问题是LE的重要组成部分，除了免疫学、神经系统症状和脑成像参数外，还代表着疾病严重程度、进展及治疗反应的重要生物标志物。因此，基于证据的神经心理学评估对于疑似或确诊边缘叶脑炎患者的诊断检查、治疗决策以及疾病和治疗监测至关重要。
