PT - JOURNAL ARTICLE AU - Thaler, Franziska S. AU - Zimmermann, Luise AU - Kammermeier, Stefan AU - Strippel, Christine AU - Ringelstein, Marius AU - Kraft, Andrea AU - Sühs, Kurt-Wolfram AU - Wickel, Jonathan AU - Geis, Christian AU - Markewitz, Robert AU - Urbanek, Christian AU - Sommer, Claudia AU - Doppler, Kathrin AU - Penner, Loana AU - Lewerenz, Jan AU - Rößling, Rosa AU - Finke, Carsten AU - Prüss, Harald AU - Melzer, Nico AU - Wandinger, Klaus-Peter AU - Leypoldt, Frank AU - Kümpfel, Tania AU - on behalf of the German Network for Research on Autoimmune Encephalitis (GENERATE) TI - Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis AID - 10.1212/NXI.0000000000001088 DP - 2021 Nov 01 TA - Neurology - Neuroimmunology Neuroinflammation PG - e1088 VI - 8 IP - 6 4099 - http://nn.neurology.org/content/8/6/e1088.short 4100 - http://nn.neurology.org/content/8/6/e1088.full SO - Neurol Neuroimmunol Neuroinflamm2021 Nov 01; 8 AB - Background and Objectives To determine the real-world use of rituximab in autoimmune encephalitis (AE) and to correlate rituximab treatment with the long-term outcome.Methods Patients with NMDA receptor (NMDAR)-AE, leucine-rich glioma-inactivated-1 (LGI1)- AE, contactin-associated protein-like-2 (CASPR2)-AE, or glutamic acid decarboxylase 65 (GAD65) disease from the GErman Network for Research on AuToimmune Encephalitis who had received at least 1 rituximab dose and a control cohort of non–rituximab-treated patients were analyzed retrospectively.Results Of the 358 patients, 163 (46%) received rituximab (NMDAR-AE: 57%, CASPR2-AE: 44%, LGI1-AE: 43%, and GAD65 disease: 37%). Rituximab treatment was initiated significantly earlier in NMDAR- and LGI1-AE (median: 54 and 155 days from disease onset) compared with CASPR2-AE or GAD65 disease (median: 632 and 1,209 days). Modified Rankin Scale (mRS) scores improved significantly in patients with NMDAR-AE, both with and without rituximab treatment. Although being more severely affected at baseline, rituximab-treated patients with NMDAR-AE more frequently reached independent living (mRS score ≤2) (94% vs 88%). In LGI1-AE, rituximab-treated and nontreated patients improved, whereas in CASPR2-AE, only rituximab-treated patients improved significantly. No improvement was observed in patients with GAD65 disease. A significant reduction of the relapse rate was observed in rituximab-treated patients (5% vs 13%). Detection of NMDAR antibodies was significantly associated with mRS score improvement. A favorable outcome was also observed with early treatment initiation.Discussion We provide real-world data on immunosuppressive treatments with a focus on rituximab treatment for patients with AE in Germany. We suggest that early and short-term rituximab therapy might be an effective and safe treatment option in most patients with NMDAR-, LGI1-, and CASPR2-AE.Class of Evidence This study provides Class IV evidence that rituximab is an effective treatment for some types of AE.abs=antibodies; AE=autoimmune encephalitis; CA=cerebellar ataxia; CASPR2=contactin-associated protein-like-2; CBA=cell-based assay; GAD65=glutamic acid decarboxylase 65; GENERATE=GErman Network for Research on AuToimmune Encephalitis; IHC=immunohistochemistry; IVIG=IV immunoglobulin; LE=limbic/autoimmune encephalitis; LGI1=leucine-rich glioma-inactivated-1; mRS=Modified Rankin Scale; NMDAR=NMDA receptor; RIA=radioimmunoassay; SPS=stiff-person syndrome