Schroeder Christoph, Back Claude, Koc Ümmügülsüm, Strassburger-Krogias Katrin, Reinacher-Schick Anke, Gold Ralf, Haghikia Aiden
Department of Neurology of the Ruhr-University Bochum at St. Josef Hospital Bochum, Germany.
Centre Hospitalier Emile Mayrisch, Esch-Alzette, Luxembourg.
Clin Neurol Neurosurg. 2018 Sep;172:24-26. doi: 10.1016/j.clineuro.2018.06.005. Epub 2018 Jun 23.
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.
自发现以来，抗N-甲基-D-天冬氨酸受体脑炎现已成为一种公认的神经炎症性疾病，针对该疾病已成功提出了多种免疫抑制策略。最常用的治疗方法包括大剂量皮质类固醇以及血浆置换程序（PLEX），随后使用口服免疫抑制剂，如硫唑嘌呤，或通过抗CD20单克隆抗体利妥昔单抗进行B细胞清除。然而，在极少数情况下，我们会遇到对口服免疫抑制剂或利妥昔单抗均无反应的患者。因此，我们最近报道了硼替佐米，一种蛋白酶体抑制剂，对一线免疫治疗无反应的患者可能是一种有效的治疗方法。特别是，浆细胞作为成熟的、不分裂的抗体分泌细胞对蛋白酶体抑制剂高度敏感。在此，我们报告一例尽管接受了PLEX和多次大剂量利妥昔单抗治疗，但仍患有严重且迁延不愈的抗NMDAR脑炎的患者。如随附视频所示，在硼替佐米治疗前及治疗后即刻的不同阶段，该患者迅速康复。
