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Early Bortezomib Therapy for Refractory Anti-NMDA Receptor Encephalitis.
Front Neurol. 2020; 11:188.FN

Abstract

Introduction: Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an increasingly recognized form of immune-mediated encephalitis. Here we present a case that represents the shortest hospitalization-to-bortezomib treatment timeline (42 days), and we believe that this is reflected in the patient's outcome with complete independence within a short timeframe. Case Report: We describe a case of anti-NMDA receptor encephalitis in an 18-year-old African American female presenting with progressive, medically refractory disease. Despite two rounds of high-dose intravenous steroids, plasma exchange, immunoglobulin administration, and rituximab for B-cell depletion, the patient failed to respond by hospital day 42 and received off-label use of the proteasome inhibitor bortezomib. During the 15 days after the bortezomib administration, the patient showed dramatic neurologic recovery that allowed her transfer out of the intensive care unit. At follow-up after 1-month, the patient reported feeling normal cognitively and showed dramatic improvement in cognitive scores.

Conclusion:

This case and literature review provide preliminary evidence that early treatment of anti-NMDA receptor encephalitis with the proteasome inhibitor bortezomib appears safe and tolerable. However, randomized trials are needed to show the efficacy and the long-term benefit.

Authors+Show Affiliations

Department of Neurology, Mayo Clinic, Jacksonville, FL, United States.Department of Neurology, Mayo Clinic, Jacksonville, FL, United States. Department of Critical Care Medicine, Mayo Clinic, Jacksonville, FL, United States.Department of Neurology, Mayo Clinic, Jacksonville, FL, United States.Department of Neurology, Mayo Clinic, Jacksonville, FL, United States.Department of Neurology, Mayo Clinic, Jacksonville, FL, United States.Department of Neurology, Mayo Clinic, Jacksonville, FL, United States. Department of Critical Care Medicine, Mayo Clinic, Jacksonville, FL, United States. Department of Neurologic Surgery, Mayo Clinic, Jacksonville, FL, United States.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

32292386

Citation

Turnbull, Marion T., et al. "Early Bortezomib Therapy for Refractory Anti-NMDA Receptor Encephalitis." Frontiers in Neurology, vol. 11, 2020, p. 188.
Turnbull MT, Siegel JL, Becker TL, et al. Early Bortezomib Therapy for Refractory Anti-NMDA Receptor Encephalitis. Front Neurol. 2020;11:188.
Turnbull, M. T., Siegel, J. L., Becker, T. L., Stephens, A. J., Lopez-Chiriboga, A. S., & Freeman, W. D. (2020). Early Bortezomib Therapy for Refractory Anti-NMDA Receptor Encephalitis. Frontiers in Neurology, 11, 188. https://doi.org/10.3389/fneur.2020.00188
Turnbull MT, et al. Early Bortezomib Therapy for Refractory Anti-NMDA Receptor Encephalitis. Front Neurol. 2020;11:188. PubMed PMID: 32292386.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Early Bortezomib Therapy for Refractory Anti-NMDA Receptor Encephalitis. AU - Turnbull,Marion T, AU - Siegel,Jason L, AU - Becker,Tara L, AU - Stephens,Alana J, AU - Lopez-Chiriboga,A Sebastian, AU - Freeman,William D, Y1 - 2020/03/27/ PY - 2020/01/13/received PY - 2020/02/27/accepted PY - 2020/4/16/entrez PY - 2020/4/16/pubmed PY - 2020/4/16/medline KW - anti-NMDA receptor encephalitis KW - autoimmune disease KW - bortezomib KW - case report KW - proteosome inhibitor SP - 188 EP - 188 JF - Frontiers in neurology JO - Front Neurol VL - 11 N2 - Introduction: Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an increasingly recognized form of immune-mediated encephalitis. Here we present a case that represents the shortest hospitalization-to-bortezomib treatment timeline (42 days), and we believe that this is reflected in the patient's outcome with complete independence within a short timeframe. Case Report: We describe a case of anti-NMDA receptor encephalitis in an 18-year-old African American female presenting with progressive, medically refractory disease. Despite two rounds of high-dose intravenous steroids, plasma exchange, immunoglobulin administration, and rituximab for B-cell depletion, the patient failed to respond by hospital day 42 and received off-label use of the proteasome inhibitor bortezomib. During the 15 days after the bortezomib administration, the patient showed dramatic neurologic recovery that allowed her transfer out of the intensive care unit. At follow-up after 1-month, the patient reported feeling normal cognitively and showed dramatic improvement in cognitive scores. Conclusion: This case and literature review provide preliminary evidence that early treatment of anti-NMDA receptor encephalitis with the proteasome inhibitor bortezomib appears safe and tolerable. However, randomized trials are needed to show the efficacy and the long-term benefit. SN - 1664-2295 UR - https://www.unboundmedicine.com/medline/citation/32292386/Early_Bortezomib_Therapy_for_Refractory_Anti_NMDA_Receptor_Encephalitis_ L2 - https://doi.org/10.3389/fneur.2020.00188 DB - PRIME DP - Unbound Medicine ER -
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