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":"lgi1抗体脑炎在年龄、性别和HLA上的临床特异性和转归。","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":"<p><strong>Background: </strong>Patients with leucine-rich glioma-inactivated 1 antibody (LGI1-Ab) encephalitis are typically elderly men that often carry human leucocyte antigen (HLA)-<i>DRB1*07:01</i> (≈90%). Herein, we aimed to investigate whether patients with atypical demographic profiles or not carrying <i>DRB1*07:01</i> have distinct clinical manifestations and outcome.</p><p><strong>Methods: </strong>Retrospective chart review of LGI1-Ab patients diagnosed at the French Reference Centre and three other European centres.</p><p><strong>Results: </strong>Among 238 patients included, median age at onset was 66 years (IQR: 60-73), 65% were male, 89% carried <i>DRB1*07:01</i> and 10% <i>DRB1*04:02</i>, 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. <i>DRB1*07:01</i> non-carriers were younger (p=0.005) and less frequently male (47%, p=0.044), while non-carriers of both <i>DRB1*07:01</i> and <i>DRB1*04:02</i> 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 <i>DRB1*07:01</i> 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).</p><p><strong>Conclusions: </strong>Age appears to be the main driver of clinical presentation, severity and outcome in LGI1-Ab encephalitis. Remarkably, <i>DRB1*07:01</i> non-carriers are younger, more commonly female and experience poorer prognosis, reflecting a distinct pathophysiology.</p>","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":"{\"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\":\"<p><strong>Background: </strong>Patients with leucine-rich glioma-inactivated 1 antibody (LGI1-Ab) encephalitis are typically elderly men that often carry human leucocyte antigen (HLA)-<i>DRB1*07:01</i> (≈90%). Herein, we aimed to investigate whether patients with atypical demographic profiles or not carrying <i>DRB1*07:01</i> have distinct clinical manifestations and outcome.</p><p><strong>Methods: </strong>Retrospective chart review of LGI1-Ab patients diagnosed at the French Reference Centre and three other European centres.</p><p><strong>Results: </strong>Among 238 patients included, median age at onset was 66 years (IQR: 60-73), 65% were male, 89% carried <i>DRB1*07:01</i> and 10% <i>DRB1*04:02</i>, 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. <i>DRB1*07:01</i> non-carriers were younger (p=0.005) and less frequently male (47%, p=0.044), while non-carriers of both <i>DRB1*07:01</i> and <i>DRB1*04:02</i> 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 <i>DRB1*07:01</i> 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).</p><p><strong>Conclusions: </strong>Age appears to be the main driver of clinical presentation, severity and outcome in LGI1-Ab encephalitis. Remarkably, <i>DRB1*07:01</i> non-carriers are younger, more commonly female and experience poorer prognosis, reflecting a distinct pathophysiology.</p>\",\"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}","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}
Clinical specificities and outcome of LGI1-antibody encephalitis according to age, sex and HLA.
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.
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.
求助内容：
应助结果提醒方式：
京公网安备 11010802042870号
