Woo-Jin Lee1, Soon-Tae Lee1, Jung-Ick Byun1, Jun-Sang Sunwoo1, Tae-Joon Kim1, Jung-Ah Lim1, Jangsup Moon1, Han Sang Lee1, Yong-Won Shin1, Keon-Joo Lee1, Soyun Kim1, Keun-Hwa Jung1, Ki-Young Jung1, Kon Chu2, Sang Kun Lee2. 1. From the Department of Neurology (W.-J.L., S.-T.L., J.-I.B., J.-S.S., T.-J.K., J.-A.L., J.M., H.S.L., Y.-W.S., K.-J.L., S.K., K.-H.J., K.-Y.J., K.C., S.K.L.), Comprehensive Epilepsy Center, Biomedical Research Institute, Seoul National University Hospital; and Program in Neuroscience (S.-T.L., J.-I.B., J.-S.S., T.-J.K., J.-A.L., J.M., H.S.L., Y.-W.S., K.-J.L., K.-H.J., K.-Y.J., K.C., S.K.L.), Neuroscience Research Institute of SNUMRC, College of Medicine, Seoul National University; Department of Neurology (J.-I.B.), Kyung Hee University Hospital at Gangdong; Soonchunhyang University School of Medicine (J.-S.S.), Seoul, South Korea. 2. From the Department of Neurology (W.-J.L., S.-T.L., J.-I.B., J.-S.S., T.-J.K., J.-A.L., J.M., H.S.L., Y.-W.S., K.-J.L., S.K., K.-H.J., K.-Y.J., K.C., S.K.L.), Comprehensive Epilepsy Center, Biomedical Research Institute, Seoul National University Hospital; and Program in Neuroscience (S.-T.L., J.-I.B., J.-S.S., T.-J.K., J.-A.L., J.M., H.S.L., Y.-W.S., K.-J.L., K.-H.J., K.-Y.J., K.C., S.K.L.), Neuroscience Research Institute of SNUMRC, College of Medicine, Seoul National University; Department of Neurology (J.-I.B.), Kyung Hee University Hospital at Gangdong; Soonchunhyang University School of Medicine (J.-S.S.), Seoul, South Korea. stemcell.snu@gmail.com sangkun2923@gmail.com.
Abstract
OBJECTIVE: To determine efficacy and safety of rituximab treatment as a second-line immunotherapy treatment for autoimmune limbic encephalitis (ALE) and to determine factors associated with functional improvement and favorable outcome following rituximab treatment. METHODS: We recruited 80 patients with ALE who were treated with rituximab as a second-line immunotherapy from the Korea Autoimmune Synaptic and Paraneoplastic Encephalitis Registry and reviewed 81 patients without rituximab as a control. We grouped patients according to the detection or type of antibodies; in addition, we evaluated clinical, laboratory, first-line immunotherapy, and rituximab treatment profiles and defined main outcomes as improvements on the modified Rankin Scale (mRS) score and a favorable mRS score (0-2) at the last follow-up. RESULTS: Functional improvement occurred more frequently in the rituximab group compared to the control group. In the rituximab group, 30 (37.5%) patients had synaptic autoantibodies, 15 (18.8%) in the paraneoplastic autoantibodies, and 35 (43.8%) were antibody-negative. The effect of rituximab was the same regardless of autoantibody status. Additional monthly rituximab therapy and partial response to first-line immunotherapies were associated with mRS score improvements, as well as favorable mRS scores. mRS scores of 4-6 as the worst neurologic status predicted an unfavorable mRS score. There were no reported serious infusion-related or infectious adverse effects of rituximab. CONCLUSIONS: Rituximab is effective and safe as a second-line immunotherapy for ALE, regardless of autoantibody status. Additional monthly rituximab therapy might potentiate the efficacy of rituximab. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that rituximab improves mRS scores for patients with autoimmune limbic encephalitis who fail first-line therapy.
OBJECTIVE: To determine efficacy and safety of rituximab treatment as a second-line immunotherapy treatment for autoimmune limbic encephalitis (ALE) and to determine factors associated with functional improvement and favorable outcome following rituximab treatment. METHODS: We recruited 80 patients with ALE who were treated with rituximab as a second-line immunotherapy from the Korea Autoimmune Synaptic and Paraneoplastic Encephalitis Registry and reviewed 81 patients without rituximab as a control. We grouped patients according to the detection or type of antibodies; in addition, we evaluated clinical, laboratory, first-line immunotherapy, and rituximab treatment profiles and defined main outcomes as improvements on the modified Rankin Scale (mRS) score and a favorable mRS score (0-2) at the last follow-up. RESULTS: Functional improvement occurred more frequently in the rituximab group compared to the control group. In the rituximab group, 30 (37.5%) patients had synaptic autoantibodies, 15 (18.8%) in the paraneoplastic autoantibodies, and 35 (43.8%) were antibody-negative. The effect of rituximab was the same regardless of autoantibody status. Additional monthly rituximab therapy and partial response to first-line immunotherapies were associated with mRS score improvements, as well as favorable mRS scores. mRS scores of 4-6 as the worst neurologic status predicted an unfavorable mRS score. There were no reported serious infusion-related or infectious adverse effects of rituximab. CONCLUSIONS:Rituximab is effective and safe as a second-line immunotherapy for ALE, regardless of autoantibody status. Additional monthly rituximab therapy might potentiate the efficacy of rituximab. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that rituximab improves mRS scores for patients with autoimmune limbic encephalitis who fail first-line therapy.
Authors: Marinos C Dalakas; Goran Rakocevic; James M Dambrosia; Harry Alexopoulos; Beverly McElroy Journal: Ann Neurol Date: 2017-08-09 Impact factor: 10.422
Authors: Anna Rada; Robert Birnbacher; Claudio Gobbi; Martin Kurthen; Albert Ludolph; Markus Naumann; Ulrike Neirich; Tim J von Oertzen; Gerhard Ransmayr; Matthias Riepe; Mareike Schimmel; Oliver Schwartz; Rainer Surges; Christian G Bien Journal: J Neurol Date: 2020-10-06 Impact factor: 4.849