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Breakthrough treatment with bortezomib for a patient with anti-NMDAR encephalitis.
Clin Neurol Neurosurg. 2018 09; 172:24-26.CN

Abstract

After its discovery, anti-N-methyl-d-aspartate receptor encephalitis is now an established neuroinflammatory disorder, for which various immune-suppressive strategies have been successfully proposed. The most commonly applied therapy includes high dose cortico-steroids, as well as plasma exchange procedures (PLEX), and subsequently either oral immunosuppressants, such as azathioprine or B-cell depletion by the anti- CD20 monoclonal antibody rituximab. However, in rare cases we are faced with patients who do not respond to either oral immunosuppressants, or rituximab. Hence, we have recently described bortezomib, a proteasome inhibitor as a potentially effective treatment in patients not responding to first-line immune-therapies. Particularly, plasma cells as mature, non-dividing antibody secreting cells are highly sensitive to proteasome inhibitors. Here, we report of a patient with severe, and prolonged anti-NMDAR encephalitis despite PLEX and repeatedly applied high dose rituximab. As documented in the accompanying video that shows the different stages before, and immediately after bortezomib therapy the patient recovered swiftly.

Authors+Show Affiliations

Department of Neurology of the Ruhr-University Bochum at St. Josef Hospital Bochum, Germany.Centre Hospitalier Emile Mayrisch, Esch-Alzette, Luxembourg.Department of Neurology of the Ruhr-University Bochum at St. Josef Hospital Bochum, Germany.Department of Neurology of the Ruhr-University Bochum at St. Josef Hospital Bochum, Germany.Section of Hematology, Oncology and Palliative Care of the Ruhr-University Bochum at St. Josef-Hospital, Bochum, Germany.Department of Neurology of the Ruhr-University Bochum at St. Josef Hospital Bochum, Germany.Department of Neurology of the Ruhr-University Bochum at St. Josef Hospital Bochum, Germany. Electronic address: Aiden.Haghikia@ruhr-uni-bochum.de.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

29960102

Citation

Schroeder, Christoph, et al. "Breakthrough Treatment With Bortezomib for a Patient With anti-NMDAR Encephalitis." Clinical Neurology and Neurosurgery, vol. 172, 2018, pp. 24-26.
Schroeder C, Back C, Koc Ü, et al. Breakthrough treatment with bortezomib for a patient with anti-NMDAR encephalitis. Clin Neurol Neurosurg. 2018;172:24-26.
Schroeder, C., Back, C., Koc, Ü., Strassburger-Krogias, K., Reinacher-Schick, A., Gold, R., & Haghikia, A. (2018). Breakthrough treatment with bortezomib for a patient with anti-NMDAR encephalitis. Clinical Neurology and Neurosurgery, 172, 24-26. https://doi.org/10.1016/j.clineuro.2018.06.005
Schroeder C, et al. Breakthrough Treatment With Bortezomib for a Patient With anti-NMDAR Encephalitis. Clin Neurol Neurosurg. 2018;172:24-26. PubMed PMID: 29960102.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Breakthrough treatment with bortezomib for a patient with anti-NMDAR encephalitis. AU - Schroeder,Christoph, AU - Back,Claude, AU - Koc,Ümmügülsüm, AU - Strassburger-Krogias,Katrin, AU - Reinacher-Schick,Anke, AU - Gold,Ralf, AU - Haghikia,Aiden, Y1 - 2018/06/23/ PY - 2017/07/07/received PY - 2018/05/24/revised PY - 2018/06/09/accepted PY - 2018/7/1/pubmed PY - 2019/12/27/medline PY - 2018/7/1/entrez KW - Anti-N-methyl-d-aspartate receptor encephalitis KW - Autoimmune diseases KW - Limbic encephalitis KW - NMDA antibodies KW - Paraneoplastic syndrome SP - 24 EP - 26 JF - Clinical neurology and neurosurgery JO - Clin Neurol Neurosurg VL - 172 N2 - After its discovery, anti-N-methyl-d-aspartate receptor encephalitis is now an established neuroinflammatory disorder, for which various immune-suppressive strategies have been successfully proposed. The most commonly applied therapy includes high dose cortico-steroids, as well as plasma exchange procedures (PLEX), and subsequently either oral immunosuppressants, such as azathioprine or B-cell depletion by the anti- CD20 monoclonal antibody rituximab. However, in rare cases we are faced with patients who do not respond to either oral immunosuppressants, or rituximab. Hence, we have recently described bortezomib, a proteasome inhibitor as a potentially effective treatment in patients not responding to first-line immune-therapies. Particularly, plasma cells as mature, non-dividing antibody secreting cells are highly sensitive to proteasome inhibitors. Here, we report of a patient with severe, and prolonged anti-NMDAR encephalitis despite PLEX and repeatedly applied high dose rituximab. As documented in the accompanying video that shows the different stages before, and immediately after bortezomib therapy the patient recovered swiftly. SN - 1872-6968 UR - https://www.unboundmedicine.com/medline/citation/29960102/Breakthrough_treatment_with_bortezomib_for_a_patient_with_anti_NMDAR_encephalitis_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0303-8467(18)30219-1 DB - PRIME DP - Unbound Medicine ER -