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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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.
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
- Anti-NMDAR encephalitis
- Autoimmune encephalitis
- Movement disorders