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

IF 4.2 2区 医学 Q2 IMMUNOLOGY
Journal of Inflammation Research Pub Date : 2025-05-30 eCollection Date: 2025-01-01 DOI:10.2147/JIR.S521219
Hao Song, Sha Xu, Bing-Qing Du, Qi-Lun Lai, Meng-Ting Cai, Hong Li, Yin Hu, Yao Ding, Mei-Ping Ding, Yin-Xi Zhang, Chun-Hong Shen
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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 (GABABR), 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 P < 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 P < 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 GABABR antibodies. Younger age at disease onset, female sex, and specific antibody profiles may be indicators of AAE.

抗体阳性自身免疫性边缘脑炎的临床特征和癫痫发作结局。
目的:自身免疫性边缘脑炎(ALE)常伴有可检测到的神经元抗体,以癫痫发作为主要临床表现。我们的目的是研究抗体阳性ALE患者的临床特征和癫痫发作结局。方法:连续招募2014年7月至2024年2月期间抗体阳性ALE患者和新发癫痫患者。收集他们的人口学、临床和临床特征以及治疗情况。分别对随访期间的癫痫发作结果及相关危险因素进行评估。结果:纳入72例患者,相关自身抗体针对富亮氨酸胶质瘤失活1 (LGI1)、γ -氨基丁酸B型受体(GABABR)和谷氨酸脱羧酶65 (GAD65)。继发性全身性强直-阵挛性发作和局灶性非运动性发作是最常见的发作符号,28例(38.9%)患者表现为多种发作类型。此外,在54例随访超过2年的患者中,16例(29.6%)经历了持续1年以上的间歇性癫痫发作。起病年龄小、特异性抗体、多种发作类型与发作持续时间长相关(均P < 0.05)。6例(11.1%)患者在随访2年后仍有癫痫发作,包括2例LGI1抗体和4例GAD65抗体。女性、年轻发病和特异性抗体谱与持续癫痫发作显著相关,提示自身免疫相关性癫痫(AAE,均P < 0.05)。结论:在抗体阳性ALE患者中,癫痫发作结果在延长的随访期间发生变化,特别是在LGI1和GABABR抗体患者中。发病年龄较小、女性和特异性抗体谱可能是AAE的指标。
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来源期刊
Journal of Inflammation Research
Journal of Inflammation Research Immunology and Microbiology-Immunology
CiteScore
6.10
自引率
2.20%
发文量
658
审稿时长
16 weeks
期刊介绍: An international, peer-reviewed, open access, online journal that welcomes laboratory and clinical findings on the molecular basis, cell biology and pharmacology of inflammation.
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