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