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

Govil-Dalela, Tuhina; Datta, Ishita; Williams, Mitchel T.

doi:10.1016/j.jneuroim.2021.577565

Cited by 4 publications

(1 citation statement)

References 16 publications

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“…As for AEs, treatment with bortezomib has been nearly exclusively reported in patients with anti-NMDAR encephalitis, in particular, in cases with severe disability who required a prolonged intensive care unit stay at the time of treatment administration, and in one patient with CASPR2 encephalitis, unsuccessfully treated with three cycles [38,39]. In the reported single cases or small case series, bortezomib was used after failure of conventional or unconventional treatments such as cyclophosphamide, rituximab, interleukin-2, or tocilizumab [39][40][41][42][43][44][45][46][47][48][49][50][51][52]. As expected, bortezomib has been shown to deplete antibody-secreting cells that were not susceptible to rituximab [52].…”

Section: Bortezomibmentioning

confidence: 99%

Treatment Options in Refractory Autoimmune Encephalitis

2022

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Autoimmune encephalitis represents a potentially treatable immune-mediated condition that is being more frequently recognized. Prompt immunotherapy is a key factor for the management of autoimmune encephalitis. First-line treatments include intravenous steroids, plasma exchange, and intravenous immunoglobulins, which can be combined in most severe cases. Rituximab and cyclophosphamide are administered as second-line agents in unresponsive cases. A minority of patients may still remain refractory, thus representing a major clinical challenge. In these cases, treatment strategies are controversial, and no guidelines exist. Treatments proposed for refractory autoimmune encephalitis include (1) cytokine-based drugs (such as tocilizumab, interleukin-2/basiliximab, anakinra, and tofacitinib); (2) plasma cell-depleting agents (such as bortezomib and daratumumab); and (3) treatments targeting intrathecal immune cells or their trafficking through the blood-brain barrier (such as intrathecal methotrexate and natalizumab). The efficacy evidence of these drugs is mostly based on case reports or small case series, with few reported controlled studies or systematic reviews. The aim of the present review is to summarize the current evidence and related methodological issues in the use of these drugs for the treatment of refractory autoimmune encephalitis.

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