Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, West Wing, John Radcliffe Hospital, Oxford OX3 9DU, UK.
Lancet Neurol. 2011 Aug;10(8):759-72. doi: 10.1016/S1474-4422(11)70096-5.
Several CNS disorders associated with specific antibodies to ion channels, receptors, and other synaptic proteins have been recognised over the past 10 years, and can be often successfully treated with immunotherapies. Antibodies to components of voltage-gated potassium channel complexes (VGKCs), NMDA receptors (NMDARs), AMPA receptors (AMPARs), GABA type B receptors (GABA(B)Rs), and glycine receptors (GlyRs) can be identified in patients and are associated with various clinical presentations, such as limbic encephalitis and complex and diffuse encephalopathies. These diseases can be associated with tumours, but they are more often non-paraneoplastic, and antibody assays can help with diagnosis. The new specialty of immunotherapy-responsive CNS disorders is likely to expand further as more antibody targets are discovered. Recent findings raise many questions about the classification of these diseases, the relation between antibodies and specific clinical phenotypes, the relative pathological roles of serum and intrathecal antibodies, the mechanisms of autoantibody generation, and the development of optimum treatment strategies.
过去 10 年来，人们已经认识到几种与离子通道、受体和其他突触蛋白的特定抗体相关的中枢神经系统疾病，并且这些疾病通常可以通过免疫疗法得到有效治疗。电压门控钾通道复合物（VGKCs）、NMDA 受体（NMDARs）、AMPA 受体（AMPARs）、GABA 型 B 受体（GABA(B)Rs）和甘氨酸受体（GlyRs）的抗体可在患者中检测到，与各种临床表现相关，如边缘性脑炎和复杂弥漫性脑病。这些疾病可能与肿瘤有关，但更常见的是非副肿瘤性的，抗体检测有助于诊断。随着更多抗体靶标的发现，免疫治疗反应性中枢神经系统疾病这一新的专业领域可能会进一步扩展。最近的发现提出了许多关于这些疾病的分类、抗体与特定临床表型的关系、血清和鞘内抗体的相对病理作用、自身抗体产生的机制以及最佳治疗策略的发展等问题。
