Bortezomib treatment for severe refractory anti-NMDA receptor encephalitis

Annals of Clinical and Translational Neurology
Yong-Won ShinKon Chu

Abstract

To evaluate the therapeutic potential of bortezomib, a proteasome inhibitor that target plasma cells, in order to revive stalled recovery in patients with anti-N-methyl-d-aspartate (NMDA) receptor encephalitis who remain bedridden even after aggressive immunotherapy. We consecutively enrolled patients with anti-NMDA receptor encephalitis who remained bedridden after first-line immunotherapy (steroids and intravenous immunoglobulin), second-line immunotherapy (rituximab), and tocilizumab treatment, and treated them with subcutaneous bortezomib. Clinical response, functional recovery, and changes in antibody titer in the serum and cerebrospinal fluid were measured. Before the bortezomib treatment, the five patients with severe refractory anti-NMDA receptor encephalitis were in a vegetative state. During the 8 months of follow-up period, three patients improved to minimally conscious states within 2 months of bortezomib treatment, one failed to improve from a vegetative state. However, no patient achieved functional recovery as measured by the modified Rankin Scale score (mRS). Three patients advanced to a cyclophosphamide with bortezomib and dexamethasone regimen, which only resulted in additional adverse events, without mRS impr...Continue Reading

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Citations

Apr 26, 2019·Current Opinion in Infectious Diseases·Arun Venkatesan
Jul 30, 2020·Nature Reviews. Neurology·Bo SunSarosh R Irani
Oct 21, 2019·Journal of Neurology·Franziska ScheibeAndreas Meisel
Apr 16, 2020·Frontiers in Neurology·Marion T TurnbullWilliam D Freeman
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Jan 3, 2021·Pediatrics in Review·Sarah PradhanAllayne Stephans
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Apr 27, 2021·Neurologic Clinics·Pooja Raibagkar, Anil Ramineni
May 12, 2021·Journal of Neuroimmunology·Alessandro DinotoPaolo Manganotti
May 25, 2021·Journal of the Intensive Care Society·Dustin AndersonPeter G Brindley
Jul 25, 2021·Neurology. Neuroimmunology and Neuroinflammation·Margherita NosadiniRussell C Dale
Aug 17, 2021·The Journal of Obstetrics and Gynaecology Research·Jiaxin GuRuiqi Duan

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Methods Mentioned

BETA
fluorescence assay

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