Refractory NMDA-receptor encephalitis in a teenager: A novel use of Bortezomib

Abstract

We report the case of a 14-year-old girl who was diagnosed with N-methyl-D-aspartate (NMDA)-receptor encephalitis, with severe features and autonomic instability, requiring intensive care unit admission. She had poor clinical response to the first-line therapies, she was then started on Rituximab and 6 cycles of Cytoxan infusion. She responded to Bortezomib therapy within 2 weeks of initiation, and with long-term sustenance of improvement in the long-term, also demonstrated by an improvement in NMDA titers. As far as we know, this is the first report of use of Bortezomib therapy in a child with refractory NMDA-receptor encephalitis.