Clinical specificities and outcome of LGI1-antibody encephalitis according to age, sex and HLA.

IF 7.5 1区医学 Q1 CLINICAL NEUROLOGY

Journal of Neurology, Neurosurgery, and Psychiatry Pub Date : 2025-10-15 DOI:10.1136/jnnp-2025-335960

Lucia Campetella, Macarena Villagrán-García, Antonio Farina, Marine Villard, Marie Benaiteau, Alberto Vogrig, Raffaele Iorio, Nicolás Lundahl Ciano-Petersen, Dimitri Psimaras, Vincent Navarro, Vicente Peris Sempere, David Gonçalves, Géraldine Picard, Véronique Rogemond, Bastien Joubert, Emmanuel Mignot, Jérôme Honnorat, Sergio Muñiz-Castrillo

{"title":"Clinical specificities and outcome of LGI1-antibody encephalitis according to age, sex and HLA.","authors":"Lucia Campetella, Macarena Villagrán-García, Antonio Farina, Marine Villard, Marie Benaiteau, Alberto Vogrig, Raffaele Iorio, Nicolás Lundahl Ciano-Petersen, Dimitri Psimaras, Vincent Navarro, Vicente Peris Sempere, David Gonçalves, Géraldine Picard, Véronique Rogemond, Bastien Joubert, Emmanuel Mignot, Jérôme Honnorat, Sergio Muñiz-Castrillo","doi":"10.1136/jnnp-2025-335960","DOIUrl":null,"url":null,"abstract":"Background: Patients with leucine-rich glioma-inactivated 1 antibody (LGI1-Ab) encephalitis are typically elderly men that often carry human leucocyte antigen (HLA)-DRB1*07:01 (≈90%). Herein, we aimed to investigate whether patients with atypical demographic profiles or not carrying DRB1*07:01 have distinct clinical manifestations and outcome.Methods: Retrospective chart review of LGI1-Ab patients diagnosed at the French Reference Centre and three other European centres.Results: Among 238 patients included, median age at onset was 66 years (IQR: 60-73), 65% were male, 89% carried DRB1*07:01 and 10% DRB1*04:02, another known secondary HLA association. We identified three age groups (young, typical, old) based on percentiles of age distribution. Young (≤51 years) patients were less commonly male (35%, p=0.004), while faciobrachial dystonic seizures (FBDS; 65%, p=0.047) and hyponatraemia (64%, p=0.046) were more frequent in old (≥79 years) patients. Old patients experienced poor outcome (modified Rankin Scale [mRS] >2 at last follow-up) more frequently (64%, p<0.001). There were no significant differences between males and females. DRB1*07:01 non-carriers were younger (p=0.005) and less frequently male (47%, p=0.044), while non-carriers of both DRB1*07:01 and DRB1*04:02 experienced poor outcome more commonly (64%, p=0.005). Older age (adjusted OR: 1.08, 95% CI [1.02 to 1.14], p=0.008), higher mRS at nadir (4.22 [2.46-7.24], p<0.001) and DRB1*07:01 non-carrier status (8.39 [1.88-37.44], p=0.005) were independently associated with poor outcome. Moreover, older age (1.08 [1.04-1.11], p<0.001), FBDS (2.20 [1.17-4.13], p=0.014) and hyponatraemia (2.30 [1.22-4.34], p=0.010) were associated with severe encephalitis (mRS >3 at nadir).Conclusions: Age appears to be the main driver of clinical presentation, severity and outcome in LGI1-Ab encephalitis. Remarkably, DRB1*07:01 non-carriers are younger, more commonly female and experience poorer prognosis, reflecting a distinct pathophysiology.","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":7.5000,"publicationDate":"2025-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Neurology, Neurosurgery, and Psychiatry","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1136/jnnp-2025-335960","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}

引用次数: 0

Abstract

Background: Patients with leucine-rich glioma-inactivated 1 antibody (LGI1-Ab) encephalitis are typically elderly men that often carry human leucocyte antigen (HLA)-DRB1*07:01 (≈90%). Herein, we aimed to investigate whether patients with atypical demographic profiles or not carrying DRB1*07:01 have distinct clinical manifestations and outcome.

Methods: Retrospective chart review of LGI1-Ab patients diagnosed at the French Reference Centre and three other European centres.

Results: Among 238 patients included, median age at onset was 66 years (IQR: 60-73), 65% were male, 89% carried DRB1*07:01 and 10% DRB1*04:02, another known secondary HLA association. We identified three age groups (young, typical, old) based on percentiles of age distribution. Young (≤51 years) patients were less commonly male (35%, p=0.004), while faciobrachial dystonic seizures (FBDS; 65%, p=0.047) and hyponatraemia (64%, p=0.046) were more frequent in old (≥79 years) patients. Old patients experienced poor outcome (modified Rankin Scale [mRS] >2 at last follow-up) more frequently (64%, p<0.001). There were no significant differences between males and females. DRB1*07:01 non-carriers were younger (p=0.005) and less frequently male (47%, p=0.044), while non-carriers of both DRB1*07:01 and DRB1*04:02 experienced poor outcome more commonly (64%, p=0.005). Older age (adjusted OR: 1.08, 95% CI [1.02 to 1.14], p=0.008), higher mRS at nadir (4.22 [2.46-7.24], p<0.001) and DRB1*07:01 non-carrier status (8.39 [1.88-37.44], p=0.005) were independently associated with poor outcome. Moreover, older age (1.08 [1.04-1.11], p<0.001), FBDS (2.20 [1.17-4.13], p=0.014) and hyponatraemia (2.30 [1.22-4.34], p=0.010) were associated with severe encephalitis (mRS >3 at nadir).

Conclusions: Age appears to be the main driver of clinical presentation, severity and outcome in LGI1-Ab encephalitis. Remarkably, DRB1*07:01 non-carriers are younger, more commonly female and experience poorer prognosis, reflecting a distinct pathophysiology.

查看原文本刊更多论文

lgi1抗体脑炎在年龄、性别和HLA上的临床特异性和转归。

背景：富含亮氨酸的胶质瘤失活1抗体（LGI1-Ab）脑炎患者多为老年男性，常携带人白细胞抗原(HLA)-DRB1*07:01（≈90%）。在此，我们旨在研究非典型人口统计学特征或不携带DRB1*07:01的患者是否具有不同的临床表现和结果。方法：回顾性分析在法国参考中心和其他三个欧洲中心诊断的LGI1-Ab患者的图表。结果：238例患者中位发病年龄为66岁（IQR: 60-73）， 65%为男性，89%携带DRB1*07:01, 10%携带DRB1*04:02，为已知的另一种继发性HLA关联。我们根据年龄分布的百分位数确定了三个年龄组（年轻、典型、老年）。年轻（≤51岁）患者中男性较少（35%,p=0.004），而老年（≥79岁）患者中面肱肌张力障碍发作（FBDS; 65%, p=0.047）和低钠血症（64%,p=0.046）更为常见。老年患者预后较差（末次随访修正Rankin量表[mRS] >2）的发生率较高（64%,pDRB1*07:01），非携带pDRB1*07:01的患者为年轻人（p=0.005），男性发生率较低（47%,p=0.044），非携带DRB1*07:01和DRB1*04:02的患者预后较差（64%,p=0.005）。年龄较大（调整后OR: 1.08, 95% CI [1.02 ~ 1.14], p=0.008）、最低时较高的mRS（4.22[2.46 ~ 7.24]）、pDRB1*07:01非携带者状态（8.39 [1.88 ~ 37.44],p=0.005）与预后不良独立相关。年龄越大（1.08[1.04-1.11]，最低点为p3）。结论：年龄似乎是LGI1-Ab脑炎的临床表现、严重程度和预后的主要驱动因素。值得注意的是，DRB1*07:01非携带者较年轻，多为女性，预后较差，反映了独特的病理生理。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

Journal of Neurology, Neurosurgery, and Psychiatry 医学-精神病学

CiteScore

15.70

自引率

1.80%

发文量

888

审稿时长

6 months

期刊介绍： The Journal of Neurology, Neurosurgery & Psychiatry (JNNP) aspires to publish groundbreaking and cutting-edge research worldwide. Covering the entire spectrum of neurological sciences, the journal focuses on common disorders like stroke, multiple sclerosis, Parkinson’s disease, epilepsy, peripheral neuropathy, subarachnoid haemorrhage, and neuropsychiatry, while also addressing complex challenges such as ALS. With early online publication, regular podcasts, and an extensive archive collection boasting the longest half-life in clinical neuroscience journals, JNNP aims to be a trailblazer in the field.