Dementia Research Centre, National Hospital for Neurology and Neurosurgery, Institute of Neurology, , London, UK.
J Neurol Neurosurg Psychiatry. 2014 Jun;85(6):625-30. doi: 10.1136/jnnp-2013-305218. Epub 2013 Jun 11.
Voltage-gated potassium channel (VGKC)-complex antibodies can be associated with a range of immunotherapy-responsive clinical presentations including limbic encephalitis, Morvan's syndrome and acquired neuromyotonia. However, there are patients with positive levels in whom the significance is uncertain.
To evaluate the clinical significance associated with positive (>100 pM) VGKC-complex antibodies.
Over a 4-year period, 1053 samples were sent for testing of which 55 were positive. The clinical presentations, final diagnoses and responses to immunotherapies, when given, were assessed retrospectively and the likelihood of autoimmunity was categorised as definite, possible, unlikely or undetermined (modified from Zuliani et al 2012).
Only 4 of the 32 patients with low-positive (100-400 pM) levels were considered definitely autoimmune, 3 with peripheral nerve hyperexcitability and 1 with a thymoma; 3 were given immunotherapies. Of the remaining 28 with low-positive levels, 13 (3 of whom had tumours) were considered possibly autoimmune, and 15 were unlikely or undetermined; 1 was given immunotherapy unsuccessfully. Of the 23 patients with high-positive (>400 pM) levels, 12 were given immunotherapies, 11 of whom showed a good response. 11 were considered definitely autoimmune, 10 with limbic encephalitis (antibody specificity: 5 LGI1, 1 contactin2, 2 negative, 2 untested) and 1 with a tumour. In the remaining 12, autoimmunity was considered possible (n=9; most had not received immunotherapies), or unlikely (n=3).
As antibody testing becomes more widely available, and many samples are referred from patients with less clear-cut diagnoses, it is important to assess the utility of the results. VGKC-complex antibodies in the range of 100-400 pM (0.1-0.4 nM) were considered clinically relevant in rare conditions with peripheral nerve hyperexcitability and appeared to associate with tumours (12.5%). By contrast high-positive (>400 pM; >0.4 nM) levels were considered definitely (38%) or possibly (49%) clinically relevant, but not all patients had a 'classical' limbic encephalitis and some did not receive immunotherapies.
电压门控钾通道 (VGKC)- 复合物抗体可与一系列免疫治疗反应的临床特征相关，包括边缘性脑炎、莫旺综合征和获得性肌强直。然而，有一些患者的抗体水平呈阳性，但意义尚不确定。
评估阳性 (>100 pM) VGKC 复合物抗体的临床意义。
在 4 年期间，共检测了 1053 个样本，其中 55 个样本呈阳性。回顾性评估了患者的临床表现、最终诊断和免疫治疗反应（如果给予的话），并根据（Zuliani 等人，2012 年）将自身免疫的可能性分为明确、可能、不太可能或不确定。
只有 4 名低阳性（100-400 pM）水平的患者被认为是明确的自身免疫性疾病，3 名患者为周围神经兴奋性过高，1 名患者为胸腺瘤；3 名患者接受了免疫治疗。在其余 28 名低阳性水平的患者中，13 名（其中 3 名患者有肿瘤）被认为可能是自身免疫性疾病，15 名不太可能或不确定；1 名患者接受免疫治疗无效。在 23 名高阳性 (>400 pM) 水平的患者中，12 名患者接受了免疫治疗，其中 11 名患者反应良好。11 名患者被认为是明确的自身免疫性疾病，其中 10 名患者患有边缘性脑炎（抗体特异性：5 名 LGI1，1 名 contactin2，2 名阴性，2 名未检测），1 名患者患有肿瘤。在其余 12 名患者中，自身免疫被认为是可能的（n=9；大多数患者未接受免疫治疗），或不太可能（n=3）。
随着抗体检测的广泛应用，越来越多的患者来自诊断不明确的患者，评估检测结果的实用性非常重要。在周围神经兴奋性过高和肿瘤等罕见情况下，100-400 pM（0.1-0.4 nM）范围内的 VGKC 复合物抗体被认为具有临床相关性（12.5%）。相比之下，高阳性 (>400 pM；>0.4 nM) 水平被认为具有明确（38%）或可能（49%）的临床相关性，但并非所有患者都有“典型”的边缘性脑炎，且并非所有患者都接受了免疫治疗。
