自身免疫性边缘脑炎 (ALE) 是一种异质性疾病，与靶向细胞外 (ALEextra) 表位、细胞内 (ALEintra) 表位、抗谷氨酸脱羧酶 65 ALE (ALEGAD65) 和无可检测抗体的 ALE (ALEabneg) 的抗体相关。通过流式细胞术对细胞参数进行分析，并结合来自 148 名 ALE 患者（33 名 ALEextra、12 名 ALEintra、28 名 ALE-GAD65、37 名 ALEabneg）的血液和脑脊液 (CSF) 中的可溶性参数，结果表明，相比之下，神经炎症（51 名复发缓解型 MS 患者 (RRMS)）和神经退行性疾病（34 名阿尔茨海默病患者 (AD)）的典型例子揭示了 ALE 亚组中离散的免疫特征。 ALE 亚型特异性标记物的鉴定有助于对罕见的 ALE 相关肿瘤进行分类，这可能会促进临床实践中的进一步诊断工作。 ALEintra 表现出神经炎症的特征，而 ALEextra 则表现出神经炎症和神经变性的特征。此外，ALEGAD65 和 ALEabneg 缺乏炎症标志。这可能解释了 ALEGAD65 和 ALEabneg 中抗炎治疗方案的低功效。版权所有 © 2024。由 Elsevier Inc. 出版。

Autoimmune limbic encephalitis (ALE) represents a heterogeneous disease associated with antibodies targeting extracellular (ALEextra) epitopes, intracellular (ALEintra) epitopes, anti-glutamic acid decarboxylase65 ALE (ALEGAD65), and ALE without detectable antibodies (ALEabneg). Combining analysis of cellular parameters, investigated by flow cytometry, and soluble parameters in the blood and cerebrospinal fluid (CSF) from a large cohort of 148 ALE patients (33 ALEextra, 12 ALEintra, 28 ALE-GAD65 37 ALEabneg) revealed, that in comparison to paradigmatic examples for neuro-inflammatory (51 relapsing remitting MS patients (RRMS)), and neuro-degenerative (34 Alzheimer's disease patients (AD)) diseases revealed discrete immune signatures in ALE subgroups. Identification of ALE-subtype specific markers facilitated classification of rare ALE-associated tumors, which may prompt further diagnostic efforts in clinical practice. While ALEintra exhibited features of neuro-inflammation, ALEextra displayed features of neuro-inflammation as well as neuro-degeneration. Moreover, ALEGAD65 and ALEabneg lacked hallmarks of inflammation. This may explain the low efficacy of anti-inflammatory treatment regimens in ALEGAD65 and presumably also ALEabneg.Copyright © 2024. Published by Elsevier Inc.