From the French Reference Center for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (L.C., A.F., M.V.-G., M.V., M.B., A.V., G.P., V.R., B.J., J.H., S.M.-C.), Hospices Civils de Lyon; MeLiS - UCBL-CNRS UMR 5284 - INSERM U1314 (L.C., A.F., M.V.-G., M.B., A.V., B.J., J.H., S.M.-C.), Université Claude Bernard Lyon 1, France; Department of Neuroscience (A.F.), Psychology, Pharmacology and Child Health, University of Florence, Italy; Department of Biostatistics (N.T.), Hospices Civils de Lyon, France; Clinical Neurology (A.V.), Santa Maria Della Misericordia University Hospital, Azienda Sanitaria Universitaria Friuli Centrale (ASU FC); Department of Medicine (DAME) (A.V.), University of Udine, Italy; Neurology Department 2-Mazarin (D.P.), Hôpitaux Universitaires La Pitié Salpêtrière-Charles Foix, APHP; Brain and Spinal Cord Institute (D.P.), INSERM U1127/CNRS UMR 7255, Université Pierre-et-Marie-Curie, Universités Sorbonnes, Paris; Neurology Department (M.R.), Hôpital Pierre Paul Riquet, CHU de Toulouse; Neurology Department (E.C.), Centre Hospitalier Universitaire Gabriel Montpied, Clermont-Ferrand; Neurology Department (C.M.), Centre Hospitalier Universitaire de Bordeaux; Immunology Department (D.G.), Hôpital Lyon Sud, Hospices Civils de Lyon, France; and Stanford Center for Sleep Sciences and Medicine (S.M.-C.), Stanford University, Palo Alto, CA.
Neurol Neuroimmunol Neuroinflamm. 2024 May;11(3):e200228. doi: 10.1212/NXI.0000000000200228. Epub 2024 Apr 11.
Relapses occur in 15%-25% of patients with leucine-rich glioma-inactivated 1 antibody (LGI1-Ab) autoimmune encephalitis and may cause additional disability. In this study, we clinically characterized the relapses and identified factors predicting their occurrence.
This is a retrospective chart review of patients with LGI1-Ab encephalitis diagnosed at our center between 2005 and 2022. Relapse was defined as worsening of previous or appearance of new symptoms after at least 3 months of clinical stabilization.
Among 210 patients, 30 (14%) experienced a total of 33 relapses. The median time to first relapse was 23.9 months (range: 4.9-110.1, interquartile range [IQR]: 17.8). The CSF was inflammatory in 11/25 (44%) relapses, while LGI1-Abs were found in the serum in 16/24 (67%) and in the CSF in 12/26 (46%); brain MRI was abnormal in 16/26 (62%) relapses. Compared with the initial episode, relapses manifested less frequently with 3 or more symptoms (4/30 patients, 13% vs 28/30, 93%; < 0.001) and had lower maximal modified Rankin scale (mRS) score (median 3, range: 2-5, IQR: 1 vs 3, range: 2-5, IQR: 0; = 0.001). The median mRS at last follow-up after relapse (2, range: 0-4, IQR: 2) was significantly higher than after the initial episode (1, range: 0-4, IQR: 1; = 0.005). Relapsing patients did not differ in their initial clinical and diagnostic features from 85 patients without relapse. Nevertheless, residual cognitive dysfunction after the initial episode (hazard ratio:13.8, 95% confidence interval [1.5; 129.5]; = 0.022) and no administration of corticosteroids at the initial episode (hazard ratio: 4.8, 95% confidence interval [1.1; 21.1]; = 0.036) were significantly associated with an increased risk of relapse.
Relapses may occur years after the initial encephalitis episode and are usually milder but cause additional disability. Corticosteroid treatment reduces the risk of future relapses, while patients with residual cognitive dysfunction after the initial episode have an increased relapse risk.
富亮氨酸胶质瘤失活 1 型抗体（LGI1-Ab）自身免疫性脑炎患者中有 15%-25%会出现复发，且可能导致额外的残疾。本研究旨在对复发情况进行临床特征描述，并确定其发生的预测因素。
本研究为回顾性图表分析，纳入 2005 年至 2022 年在我院诊断为 LGI1-Ab 脑炎的患者。复发定义为临床稳定至少 3 个月后出现先前症状恶化或新症状出现。
在 210 例患者中，30 例（14%）共发生 33 次复发。首次复发的中位时间为 23.9 个月（范围：4.9-110.1，四分位距 [IQR]：17.8）。25 次复发中有 11 次（44%）脑脊液呈炎症表现，24 次中有 16 次（67%）血清 LGI1-Ab 阳性，26 次中有 12 次（46%）脑脊液 LGI1-Ab 阳性；26 次复发中有 16 次（62%）脑 MRI 异常。与首次发作相比，复发时出现 3 个或更多症状的频率更低（4/30 例，13% vs 28/30 例，93%；<0.001），最大改良 Rankin 量表（mRS）评分更低（中位数 3，范围：2-5，IQR：1 vs 3，范围：2-5，IQR：0；=0.001）。复发后末次随访的 mRS 中位数（2，范围：0-4，IQR：2）显著高于首次发作后（1，范围：0-4，IQR：1；=0.005）。与未复发的 85 例患者相比，复发患者在初始临床和诊断特征方面无差异。然而，首次发作后存在认知功能障碍（风险比：13.8，95%置信区间 [1.5；129.5]；=0.022）和首次发作时未使用皮质类固醇（风险比：4.8，95%置信区间 [1.1；21.1]；=0.036）与复发风险增加显著相关。
复发可能在首次脑炎发作多年后发生，且通常较轻微，但会导致额外的残疾。皮质类固醇治疗可降低未来复发的风险，而首次发作后存在认知功能障碍的患者复发风险增加。
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