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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
Predictors and Clinical Characteristics of Relapses in LGI1-Antibody Encephalitis
Lucia Campetella1, Antonio Farina1, Macarena Villagrán-García1
1From 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.
Background And Objectives:
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.
Methods:
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.
Results:
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%; p < 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; p = 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; p = 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]; p = 0.022) and no administration of corticosteroids at the initial episode (hazard ratio: 4.8, 95% confidence interval [1.1; 21.1]; p = 0.036) were significantly associated with an increased risk of relapse.
Discussion:
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.
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