Refractory anti-NMDAR encephalitis successfully treated with bortezomib and associated movements disorders controlled with tramadol: a case report with literature review

A Correction to this article was published on 08 January 2021

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Abstract

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a potentially fatal autoimmune disease, characterized by autoantibody-mediated neurotransmission impairment in multiple brain locations. The course of this condition often comprises altered mental status, autonomic dysfunctions, refractory seizures and hyperkinetic movement disorders. Available disease-modifying therapies include corticosteroids, i.v. immunoglobulins, plasma exchange, rituximab and cyclophosphamide. In a subgroup of patients not responding to B-cell depletion, bortezomib, a proteasome inhibitor, has shown promising evidence of efficacy. The time course of recovery from acute phase may be very slow (weeks/months), and only few data are available in literature about the concurrent management of encephalitis-associated movement disorders. We report a case of severe anti-NMDAR encephalitis in a 29-year-old woman, not responsive to first- and second-line treatments, with persistent involuntary motor manifestations. Starting three months after symptom onset, four cycles of bortezomib have been administered; subsequently we observed a progressive improvement of neurological status. Meanwhile, motor manifestations were controlled after the administration of tramadol, a non-competitive NMDA receptor antagonist.

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Authors

Contributions

S.M.L. and M.V. equally contributed to paper conception, data collection, analysis and interpretation, literature review and paper drafting. G.C. contributed to data collection, analysis and interpretation, literature review and paper drafting. R.F., G.F.F., M.A.V., A.Ge. and A.Gi. contributed to data collection, analysis and critical review. V.M., S.C., P.B., M.M. and J.P. supervised data collection and performed critical review. M.F. and F.M. contributed to paper conception, supervised data analysis and interpretation, and critically reviewed the paper.

Corresponding author

Correspondence to Massimo Filippi.

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No relevant competing interest was declared by the Authors.

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Informed consent for publication was obtained verbally from the spouse for the usage of clinical data in anonymized fashion. The consent was audio-recorded in the presence of an independent witness.

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The Ethics Committee of San Raffaele Scientific Institute waived the need for ethics approval for publication of case reports.

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Lazzarin, S.M., Vabanesi, M., Cecchetti, G. et al. Refractory anti-NMDAR encephalitis successfully treated with bortezomib and associated movements disorders controlled with tramadol: a case report with literature review. J Neurol 267, 2462–2468 (2020). https://doi.org/10.1007/s00415-020-09988-w

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Keywords

  • Anti-NMDAR encephalitis
  • Autoimmune encephalitis
  • Bortezomib
  • EEG
  • Movement disorders