Department of Neurology, Erasmus Medical Center, Rotterdam, The Netherlands.; Department of Neurology, Haga Teaching Hospital, The Hague, The Netherlands.
Department of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands..
Autoimmun Rev. 2016 Oct;15(10):970-4. doi: 10.1016/j.autrev.2016.07.018. Epub 2016 Jul 30.
A wide variety of clinical syndromes has been associated with antibodies to voltage-gated potassium channels (VGKCs). Six years ago, it was discovered that patients do not truly have antibodies to potassium channels, but to associated proteins. This enabled the distinction of three VGKC-positive subgroups: anti-LGI1 patients, anti-Caspr2 patients and VGKC-positive patients lacking both antibodies. Patients with LGI1-antibodies have a limbic encephalitis, often with hyponatremia, and about half of the patients have typical faciobrachial dystonic seizures. Caspr2-antibodies cause a more variable syndrome of peripheral or central nervous system symptoms, almost exclusively affecting older males. Immunotherapy seems to be beneficial in patients with antibodies to LGI1 or Caspr2, stressing the need for early diagnosis. Half of the VGKC-positive patients lack antibodies to both LGI1 and Caspr2. This is a heterogeneous group of patients with a wide variety of clinical syndromes, raising the question whether VGKC-positivity is truly a marker of disease in these patients. Data regarding this issue are limited, but a recent study did not show any clinical relevance of VGKC-positivity in the absence of antibodies to LGI1 and Caspr2. The three VGKC-positive subgroups are essentially different, therefore, the lumping term 'VGKC-complex antibodies' should be abolished.
已发现多种临床综合征与电压门控钾通道（VGKC）抗体相关。六年前，人们发现患者并非真正产生针对钾通道的抗体，而是针对相关蛋白。这使得我们能够区分三种 VGKC 阳性亚组：抗 LGI1 患者、抗 Caspr2 患者和同时缺乏这两种抗体的 VGKC 阳性患者。LGI1 抗体患者患有边缘性脑炎，常伴有低钠血症，约一半患者有典型面臂部阵挛性癫痫发作。Caspr2 抗体引起外周或中枢神经系统症状更具变异性的综合征，几乎仅影响老年男性。免疫治疗似乎对 LGI1 或 Caspr2 抗体患者有益，强调了早期诊断的必要性。一半的 VGKC 阳性患者同时缺乏针对 LGI1 和 Caspr2 的抗体。这是一组具有多种临床综合征的异质性患者，这使得 VGKC 阳性是否确实是这些患者疾病的标志物这一问题受到质疑。关于这一问题的数据有限，但最近的一项研究表明，在缺乏针对 LGI1 和 Caspr2 的抗体的情况下，VGKC 阳性没有任何临床相关性。因此，这三种 VGKC 阳性亚组本质上不同，因此，应废除“VGKC 复合物抗体”这一统称。
