Bortezomib in anti-N-Methyl-d-Aspartate-Receptor (NMDA-R) encephalitis: A systematic review

Abstract

N-methyl-d-aspartate receptor (NMDAR) encephalitis is a potentially treatable condition, although a small proportion of patients remains refractory to immunotherapy. Bortezomib is a proteasome inhibitor that has a promising role in autoimmune conditions. We performed an independent PubMed search employing "Anti-N-Methyl‑D-Aspartate encephalitis AND bortezomib", including papers published between January 1st, 2007 to April 15th, 2021. Fourteen articles were included, with 29 patients. 16 patients (55,2%) had a favorable outcome after bortezomib and 11 (37,9%) patients developed side effects. Quality of studies was overall poor and future trials should aim to include more homogeneous and larger cohorts.