Extra-central nervous system target for assessment and treatment in refractory anti-N-methyl-d-aspartate receptor encephalitis.
Extra-central nervous system target for assessment and treatment in refractory anti-N-methyl-d-aspartate receptor encephalitis.
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DOI：
10.1016/j.jcrc.2016.09.016
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发表时间：
2017-02
影响因子：
3.7
通讯作者：
Nauen DW
中科院分区：
文献类型：
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作者：
Nauen DW
抗N-甲基-D-谷氨酸受体自身免疫性脑炎可发生在卵巢畸胎瘤的情况下，常对切除术有反应。在没有肿瘤的情况下出现时，可能更有可能对治疗无效，受影响的患者通常需要重症监护。为了进一步了解机制和潜在的管理，我们目前的结果进行尸检的一名年轻女子谁死于难治性自身免疫性脑炎这种类型。死亡前70天给予利妥昔单抗，死亡前37天和14天CD 19+淋巴细胞仅占血细胞的0.1%。利妥昔单抗治疗后进行了10次血浆置换。尽管如此，死亡前4天血清中存在自身抗体，表明正在进行抗体产生。海马和内侧颞叶表现出炎症，T细胞和突出的小胶质细胞参与，但没有发现浆细胞或浆母细胞，或在大脑中的任何地方，尽管广泛的搜索。淋巴结组织检查发现沿着血窦有许多浆细胞。这些发现表明，抗体产生细胞是长寿的，可能存在于淋巴组织中。持续抗体产生的意识，中枢神经系统外的网站，细胞毒性治疗的适应症，活检评估的可能性可能会导致更有效的治疗。
Anti-N-methyl-D-aspartate-type glutamate receptor autoimmune encephalitis can arise in the setting of ovarian teratoma and often responds to resection. When arising in the absence of tumor failure to respond to treatment may be more likely, and affected patients often require intensive care. To further understanding of mechanisms and potential management we present findings from an autopsy conducted on a young woman who died from refractory autoimmune encephalitis of this type. Rituximab was administered 70 days before death, and both 37 and 14 days before death CD19+ lymphocytes were only 0.1% of blood cells. 10 sessions of plasmapheresis were performed following rituximab treatment. Nonetheless, the auto-antibodies were present in serum 4 days before death, demonstrating ongoing antibody production. The hippocampus and medial temporal lobe demonstrated inflammation with T-cell and prominent microglial involvement, but no plasma cells or plasmablasts were found there, or anywhere in the brain, despite an extensive search. Examination of lymph node tissue identified many plasma cells along sinusoids. These findings demonstrate that the antibody-producing cells are long-lived and likely reside in lymphoid tissue. Awareness of continuing antibody production, the extra-CNS site, the indication for cytotoxic therapy, and the potential for biopsy assessment may lead to more effective treatment.