Massimo Filippi, Vittorio Martinelli, Sergio Colombo, Angela Genchi, Milena Mucci, Raffaella Fazio, Serena Marita Lazzarin, Marco Vabanesi, Maria Antonietta Volontè, Giordano Cecchetti, Jacopo Peccatori, Paolo Beccaria, Antonino Giordano, G. Fanelli, Fabio Minicucci, Lazzarin, S. M., Vabanesi, M., Cecchetti, G., Fazio, R., Fanelli, G. F., Volonte, M. A., Genchi, A., Giordano, A., Martinelli, V., Colombo, S., Beccaria, P., Mucci, M., Peccatori, J., Filippi, M., and Minicucci, F.
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.