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抗体阳性自身免疫性边缘叶脑炎的临床特征与癫痫发作结局

Clinical Characteristics and Seizure Outcomes in Antibody-Positive Autoimmune Limbic Encephalitis.

作者信息

Song Hao, Xu Sha, Du Bing-Qing, Lai Qi-Lun, Cai Meng-Ting, Li Hong, Hu Yin, Ding Yao, Ding Mei-Ping, Zhang Yin-Xi, Shen Chun-Hong

机构信息

Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, 310009, People's Republic of China.

Department of Neurology, Zhejiang Hospital, Hangzhou, 310013, People's Republic of China.

出版信息

J Inflamm Res. 2025 May 30;18:7055-7065. doi: 10.2147/JIR.S521219. eCollection 2025.

Abstract

PURPOSE

Autoimmune limbic encephalitis (ALE) often occurs with detectable neuronal antibodies, presenting with seizures as a prominent clinical manifestation. We aimed to investigate the clinical characteristics and seizure outcomes in a cohort with antibody-positive ALE.

METHODS

We consecutively recruited patients with antibody-positive ALE and new-onset seizures between July 2014 and February 2024. Their demographic, clinical, and paraclinical characteristics, and treatment were collected. Seizure outcomes during follow-up were evaluated respectively, as well as the associated risk factors.

RESULTS

Seventy-two patients were included, and the associated autoantibodies targeted the leucine-rich glioma-inactivated 1 (LGI1), gamma-aminobutyric acid type B receptor (GABAR), and glutamic acid decarboxylase 65 (GAD65). Secondarily generalized tonic-clonic seizures and focal non-motor seizures were the most prevalent seizure semiologies, and 28 (38.9%) patients exhibited multiple seizure types. Furthermore, among 54 patients with over two years of follow-up, 16 (29.6%) experienced intermittent seizures lasting for more than one year. Younger onset, specific antibodies, and multiple seizure types were correlated with the longer seizure duration (all < 0.05). Six (11.1%) patients continued to have seizures even after two years of follow-up, comprising two with LGI1 and four with GAD65 antibodies. Female sex, younger onset, and specific antibody profiles were significantly associated with sustained seizures, indicating autoimmune-associated epilepsy (AAE, all < 0.05).

CONCLUSION

In patients with antibody-positive ALE, seizure outcomes appeared to change over an extended follow-up period, particularly in those with LGI1 and GABAR antibodies. Younger age at disease onset, female sex, and specific antibody profiles may be indicators of AAE.

摘要

目的

自身免疫性边缘叶脑炎(ALE)常伴有可检测到的神经元抗体,癫痫发作是其突出的临床表现。我们旨在研究抗体阳性ALE队列的临床特征和癫痫发作结局。

方法

我们连续招募了2014年7月至2024年2月期间抗体阳性ALE且新发癫痫发作的患者。收集他们的人口统计学、临床和辅助检查特征以及治疗情况。分别评估随访期间的癫痫发作结局以及相关危险因素。

结果

纳入72例患者,相关自身抗体靶向富含亮氨酸的胶质瘤失活1(LGI1)、γ-氨基丁酸B型受体(GABAR)和谷氨酸脱羧酶65(GAD65)。继发性全面强直阵挛发作和局灶性非运动性发作是最常见的癫痫发作形式,28例(38.9%)患者表现出多种发作类型。此外,在54例随访超过两年的患者中,16例(29.6%)经历了持续超过一年的间歇性癫痫发作。发病年龄较小、特定抗体和多种发作类型与癫痫发作持续时间较长相关(均P<0.05)。6例(11.1%)患者即使在随访两年后仍继续有癫痫发作,其中2例为LGI1抗体阳性,4例为GAD65抗体阳性。女性、发病年龄较小和特定抗体谱与持续性癫痫发作显著相关,提示自身免疫性相关癫痫(AAE,均P<0.05)。

结论

在抗体阳性ALE患者中,癫痫发作结局在长期随访中似乎会发生变化,尤其是那些LGI1和GABAR抗体阳性的患者。发病年龄较小、女性以及特定抗体谱可能是AAE的指标。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a024/12132054/9801539f8202/JIR-18-7055-g0001.jpg

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