Buckley Camilla, Vincent Angela
University of Oxford, UK.
Nat Clin Pract Neurol. 2005 Nov;1(1):22-33. doi: 10.1038/ncpneuro0033.
Autoimmune disorders of the neuromuscular junction remain a paradigm for our understanding of autoimmunity. Since the role of autoantibodies to acetylcholine receptors in the pathogenesis of myasthenia gravis was first recognized in the 1970s, a range of antibody-mediated disorders of the neuromuscular junction have been described, each associated with an autoantibody to a specific ligand-gated receptor, voltage-gated ion channel or related protein. In addition, antibodies to a ganglionic form of acetylcholine receptor have been detected in autoimmune forms of autonomic neuropathy. In the past few years, a role for antibodies in disorders of the CNS has begun to emerge, challenging our previous concepts regarding the blood-brain barrier and the role of the humoral immune system in CNS pathology. Although it has not yet been definitively shown that these CNS conditions are antibody-mediated, the detection of the specific antibody supports a trial of immunosuppressive therapy to which many patients appear to respond. In this article, we review the roles of antibodies to receptors and ion channels in the peripheral and central nervous systems, concentrating on the recently defined autonomic and CNS conditions and on the role of antibody measurement in diagnosis and management.
神经肌肉接头自身免疫性疾病仍然是我们理解自身免疫的范例。自20世纪70年代首次认识到抗乙酰胆碱受体自身抗体在重症肌无力发病机制中的作用以来，已经描述了一系列抗体介导的神经肌肉接头疾病，每种疾病都与针对特定配体门控受体、电压门控离子通道或相关蛋白的自身抗体有关。此外，在自身免疫性自主神经病变形式中检测到了针对神经节形式乙酰胆碱受体的抗体。在过去几年中，抗体在中枢神经系统疾病中的作用开始显现，这挑战了我们之前关于血脑屏障以及体液免疫系统在中枢神经系统病理学中作用的概念。尽管尚未明确证明这些中枢神经系统疾病是由抗体介导的，但特定抗体的检测支持进行免疫抑制治疗试验，许多患者似乎对此有反应。在本文中，我们回顾了受体和离子通道自身抗体在周围和中枢神经系统中的作用，重点关注最近定义的自主神经和中枢神经系统疾病以及抗体检测在诊断和管理中的作用。
