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Refractory anti-NMDAR encephalitis successfully treated with bortezomib and associated movements disorders controlled with tramadol: a case report with literature review
Authors:Lazzarin  Serena Marita  Vabanesi   Marco  Cecchetti   Giordano  Fazio   Raffaella  Fanelli   Giovanna Franca  Volonté   Maria Antonietta  Genchi   Angela  Giordano   Antonino  Martinelli   Vittorio  Colombo   Sergio  Beccaria   Paolo  Mucci   Milena  Peccatori   Jacopo  Filippi   Massimo  Minicucci   Fabio
Affiliation:1.Neurology Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy
;2.Neurophysiology Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy
;3.General Intensive Care Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy
;4.Hematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy
;5.Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, IRCCS San Raffaele Scientific Institute, Via Olgettina, 60, 20132, Milan, Italy
;6.Vita-Salute San Raffaele University, Milan, Italy
;
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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