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抗LGI1抗体脑炎患者的临床、脑电图及影像学特征:一项多中心队列研究

Clinical, Electroencephalogram and Imaging Characteristics of Patients With Anti-LGI1 Antibody Encephalitis: A Multicenter Cohort Study.

作者信息

Zhao Yang, Yuan Yue, Wang Rong-Ze, Cui Mei, Chen Shu-Fen, Chen Ke-Liang, Li Meng-Meng, Huang Yu-Yuan, Zhang Hai-Ning, Zhang Yan, Zhao Meng, Tian Hui, Sun Li, Yu Jin-Tai

机构信息

Department of Neurology, The First Hospital of Jilin University, Changchun, Jilin Province, China.

Department of Neurology and National Center for Neurological Diseases, Huashan Hospital, State Key Laboratory of Medical Neurobiology and MOE Frontiers Center for Brain Science, Shanghai Medical College, Fudan University, Shanghai, China.

出版信息

CNS Neurosci Ther. 2025 May;31(5):e70414. doi: 10.1111/cns.70414.

DOI:10.1111/cns.70414
PMID:40322833
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12051031/
Abstract

OBJECTIVES

To summarize the clinical, electroencephalogram (EEG), and imaging characteristics of patients with anti-leucine-rich glioma-inactivated 1 autoimmune encephalitis (LGI1-AE) and provide a reference for clinical diagnosis and treatment.

METHODS

We retrospectively analyzed 88 patients diagnosed with LGI1-AE between January 2018 and April 2024 in the Department of Neurology, Huashan Hospital, Fudan University, and the First Hospital of Jilin University.

RESULTS

This retrospective study analyzed 88 patients diagnosed with LGI1-AE. The initial clinical presentation predominantly featured rapidly progressive cognitive impairment (RPCI) (51.1%) and seizures (50%). Brain magnetic resonance imaging and 18 F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) indicated predominant lesion localization in the unilateral or bilateral temporal lobe and/or basal ganglia. Abnormal EEG was observed in 66 cases (79.5%). LGI1-AE cases had increased power in the low-frequency bands (δ and θ) compared to normal controls. Low-frequency band (δ and θ) power in T3 and Fz channels was positively correlated with LGI1 antibody titers in cerebrospinal fluid (CSF). Spearman correlation analysis showed that baseline modified Rankin Scale (mRS) scores were correlated with serum antibody titers and CSF antibody titers.

CONCLUSIONS

Baseline mRS scores and low-frequency power in the frontotemporal region showed a positive correlation with anti-LGI1 antibody titers, suggesting that antibody levels may reflect disease severity in LGI1 autoimmune encephalitis. Further studies are warranted to validate these associations in prospective multicenter cohorts.

摘要

目的

总结抗富含亮氨酸胶质瘤失活1自身免疫性脑炎(LGI1-AE)患者的临床、脑电图(EEG)及影像学特征,为临床诊断和治疗提供参考。

方法

回顾性分析2018年1月至2024年4月在复旦大学附属华山医院神经内科及吉林大学第一医院确诊为LGI1-AE的88例患者。

结果

本回顾性研究分析了88例确诊为LGI1-AE的患者。初始临床表现主要为快速进展性认知障碍(RPCI)(51.1%)和癫痫发作(50%)。脑磁共振成像和18F-氟脱氧葡萄糖正电子发射断层扫描(18F-FDG PET)显示主要病变定位于单侧或双侧颞叶和/或基底节。66例(79.5%)观察到EEG异常。与正常对照相比,LGI1-AE患者低频带(δ和θ)功率增加。T3和Fz通道的低频带(δ和θ)功率与脑脊液(CSF)中LGI1抗体滴度呈正相关。Spearman相关性分析显示,基线改良Rankin量表(mRS)评分与血清抗体滴度和CSF抗体滴度相关。

结论

基线mRS评分和额颞区低频功率与抗LGI1抗体滴度呈正相关,提示抗体水平可能反映LGI1自身免疫性脑炎的疾病严重程度。有必要进行进一步研究以在前瞻性多中心队列中验证这些关联。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32b0/12051031/88a59bce4994/CNS-31-e70414-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32b0/12051031/4388b781ffd9/CNS-31-e70414-g004.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32b0/12051031/47e1f4ba301a/CNS-31-e70414-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32b0/12051031/88a59bce4994/CNS-31-e70414-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32b0/12051031/4388b781ffd9/CNS-31-e70414-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32b0/12051031/c9ea141ea697/CNS-31-e70414-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32b0/12051031/3295e08092af/CNS-31-e70414-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32b0/12051031/47e1f4ba301a/CNS-31-e70414-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32b0/12051031/88a59bce4994/CNS-31-e70414-g003.jpg

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2
Clinical characteristics and prognosis in patients with neuronal surface antibody-mediated autoimmune encephalitis: a single-center cohort study in China.中国单中心队列研究:神经元表面抗体介导自身免疫性脑炎患者的临床特征和预后。
Front Immunol. 2023 Dec 12;14:1213532. doi: 10.3389/fimmu.2023.1213532. eCollection 2023.
3
Electrolyte Imbalance in Anti-LGI1 Encephalitis: It Is Not All in Your Head.
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Neurol Neuroimmunol Neuroinflamm. 2023 Aug 17;10(6). doi: 10.1212/NXI.0000000000200155. Print 2023 Nov.
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Autoimmune encephalitis: recent clinical and biological advances.自身免疫性脑炎:最新的临床和生物学进展。
J Neurol. 2023 Aug;270(8):4118-4131. doi: 10.1007/s00415-023-11685-3. Epub 2023 Apr 28.
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Patient-derived antibodies reveal the subcellular distribution and heterogeneous interactome of LGI1.患者来源的抗体揭示 LGI1 的亚细胞分布和异质相互作用组。
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