Buckley C, Oger J, Clover L, Tüzün E, Carpenter K, Jackson M, Vincent A
Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, UK.
Ann Neurol. 2001 Jul;50(1):73-8. doi: 10.1002/ana.1097.
Limbic encephalitis (LE) is often associated with lung, thymic, or testicular tumours and antibodies to Hu, CV2, or Ma2 (Ta) antigens. In these cases, it generally has a poor prognosis. Here we describe two patients with symptoms of LE, negative for typical paraneoplastic antibodies, in whom antibodies to voltage-gated potassium channels (VGKC) were detected retrospectively in serial serum samples. Patient 1 had a thymoma recurrence, but in patient 2 no tumour has been detected in the years following presentation. Plasma exchange was effective in reducing VGKC antibody levels, with substantial improvement in mental symptoms in patient 1. In patient 2, the VGKC antibodies fell spontaneously over two years, with almost complete recovery of mental function. Although neither patient had obvious neuromyotonia at presentation, both showed excessive secretions. We suggest that patients with limbic symptoms and excessive secretions should be tested for VGKC antibodies, and, if they are present, prompt and effective immunosuppressive treatment should be considered.
边缘叶脑炎(LE)常与肺、胸腺或睾丸肿瘤以及抗Hu、CV2或Ma2(Ta)抗原的抗体相关。在这些病例中,其预后通常较差。在此,我们描述了两名有LE症状但典型副肿瘤抗体检测呈阴性的患者,在其系列血清样本中回顾性检测到了抗电压门控钾通道(VGKC)抗体。患者1出现胸腺瘤复发,但患者2在发病后的数年中未检测到肿瘤。血浆置换有效降低了VGKC抗体水平,患者1的精神症状有显著改善。在患者2中,VGKC抗体在两年内自行下降,精神功能几乎完全恢复。尽管两名患者在发病时均无明显的神经性肌强直,但均表现出分泌过多。我们建议,有边缘叶症状和分泌过多的患者应检测VGKC抗体,若存在该抗体,则应考虑及时进行有效的免疫抑制治疗。