What is the promise of Bortezomib in refractory anti-NMDA receptor encephalitis?

Bortezomib for treatment of therapy-refractory anti-NMDA receptor encephalitis

Scheibe F, Prüss H, Mengel AM, et al.

Neurology 2017; 88:366-370.



We assessed the therapeutic potential of the plasma-cell-depleting proteasome inhibitor bortezomib in severe and therapy-refractory cases of anti-NMDA receptor (anti-NMDAR) encephalitis.


Five severely affected patients with anti-NMDAR encephalitis with delayed treatment response or resistance to standard immunosuppressive and B-cell-depleting drugs (corticosteroids, IV immunoglobulins, plasma exchange, immunoadsorption, rituximab, cyclophosphamide) who required medical treatment and artificial ventilation on intensive care units were treated with 1-6 cycles of 1.3 mg/m2bortezomib. Occurrence of adverse events was closely monitored.


Bortezomib treatment showed clinical improvement or disease remission, which was accompanied by a partial NMDAR antibody titer decline in 4 of 5 patients. With respect to disease severity, addition of bortezomib to the multimodal immunosuppressive treatment regimen was associated with an acceptable safety profile.


Our study identifies bortezomib as a promising escalation therapy for severe and therapy-refractory anti-NMDAR encephalitis.

Similar article:

Behrendt V, Krogias C, Reinacher-Schick A, Gold R, Kleiter I. Bortezomib treatment for patients with anti-N-Methyl-D-Aspartase receptor encephalitis. JAMA Neurol 2016; 73:1251-1253.

Both references are cited in the neurochecklist:

Anti NMDA receptor antibody autoimmune encephalitis: management

Abstract 1

Abstract 2

By millennium – velcade.com, CC BY-SA 3.0, Link



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