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Bortezomib Treatment for Patients With Anti-N-Methyl-d-Aspartate Receptor Encephalitis ONLINE FIRST

Volker Behrendt, MD1; Christos Krogias, MD1; Anke Reinacher-Schick, MD2; Ralf Gold, MD1; Ingo Kleiter, MD1
[+] Author Affiliations
1Department of Neurology, St Josef Hospital, Ruhr University of Bochum, Bochum, Germany
2Section of Hematology and Oncology, Medical Department, St Josef Hospital, Ruhr University of Bochum, Bochum, Germany
JAMA Neurol. Published online August 15, 2016. doi:10.1001/jamaneurol.2016.2588
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This case series describes 2 women with anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis whose disease remitted with bortezomib treatment.

Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis, an autoimmune disease associated with ovarian teratoma, predominantly affects young females.1 Because antibodies against subunits of the NMDAR contribute to the pathogenesis, methods targeting humoral immunity are therapeutically efficacious.2 However, some patients have an unsatisfactory outcome after high-dose corticosteroids, apheresis therapies, or CD20-targeted B-cell depletion with rituximab.2 We describe 2 patients with severe anti-NMDAR encephalitis who received the proteasome inhibitor bortezomib (Velcade), which was well tolerated and followed by marked remission.

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Clinical Course and Treatment in Cases 1 (A) and 2 (B)

The x-axis indicates the number of months after disease onset. The y-axis indicates the anti-N-methyl-d-aspartate receptor antibody titer measured by a standard cell-based assay in the cerebrospinal fluid (CSF [triangles]) and serum (connected by dashed line). The clinical course is documented by the modified Rankin Scale (mRS) score depicted as horizontal bars below the graph. IA indicates immunoadsorption; IVIG, intravenous immunoglobulins; mPSL, methylprednisolone; and PLEX, plasma exchange.

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