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The expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathies.

机译:临床上独特的,免疫治疗反应性自身免疫性脑病的范围正在扩大。

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摘要

The autoimmune encephalopathies are a group of conditions that are associated with autoantibodies against surface neuronal proteins, which are likely to mediate the disease. They are established as a frequent cause of encephalitis. Characteristic clinical features in individual patients often allow the specificity of the underlying antibody to be confidently predicted. Antibodies against the VGKC-complex, mainly LGI1(leucine-rich glioma-inactivated 1), CASPR2 (contactin-associated protein 2), and contactin-2, and NMDA (N-methyl, D-aspartate) -receptor are the most frequently established serological associations. In the minority of cases, an underlying tumour can be responsible. Early administration of immunotherapies, and tumour removal, where it is relevant, offer the greatest chance of improvement. Prolonged courses of immunotherapies may be required, and clinical improvements often correlate well with the antibody levels. In the present article, we have summarised recent developments in the clinical and laboratory findings within this rapidly expanding field.
机译:自身免疫性脑病是与针对表面神经元蛋白的自身抗体相关的一组疾病,这些蛋白可能介导该疾病。它们被确定为脑炎的常见病因。个别患者的特征性临床特征通常可以使基础抗体的特异性得到可靠的预测。针对VGKC复合物的抗体是最常见的抗体,主要是LGI1(富含亮氨酸的神经胶质瘤灭活的1),CASPR2(接触素相关的蛋白质2)和contactin-2和NMDA(N-甲基,D-天冬氨酸)受体。建立了血清学协会。在少数情况下,可能是潜在的肿瘤。尽早进行免疫治疗和切除肿瘤是最大的改善机会。可能需要延长免疫疗法的疗程,并且临床改善通常与抗体水平密切相关。在本文中,我们总结了这个快速扩展领域中临床和实验室发现的最新进展。

著录项

  • 作者

    Irani, SR; Vincent, A;

  • 作者单位
  • 年度 2012
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  • 原文格式 PDF
  • 正文语种 eng
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