Giannoccaro Maria Pia, Crisp Sarah J, Vincent Angela
Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.
Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy.
Brain Neurosci Adv. 2018 Dec 10;2:2398212818817497. doi: 10.1177/2398212818817497. eCollection 2018 Jan-Dec.
Antibody-mediated central nervous system diseases are a relatively new area of clinical neuroscience with growing impact. Their recognition has challenged the dogma of the blood-brain barrier preventing antibody access into the central nervous system. The antibodies discovered so far are mainly against neurotransmitter receptors (e.g. -methyl-d-aspartate and glycine receptors) and ion channel-associated proteins (leucine-rich glioma inactivated protein 1 and contactin-associated protein 2) and are expressed on the surface of neuronal synapses and elsewhere. The disorders are reversible with immunotherapies that reduce antibody levels. Although rare, the identification of these disorders in clinical practice has made central nervous system autoimmune diseases a consideration in the differential diagnoses of many clinical presentations. There is still much to learn about the aetiology of the diseases and the mechanisms by which the antibodies act, the neuronal and glial changes that follow antibody-attack, and the compensatory changes that may be required to ensure good recovery.
抗体介导的中枢神经系统疾病是临床神经科学中一个相对较新的领域,其影响日益增大。它们的发现对血脑屏障阻止抗体进入中枢神经系统的传统观念提出了挑战。迄今为止发现的抗体主要针对神经递质受体(如N-甲基-D-天冬氨酸和甘氨酸受体)和离子通道相关蛋白(富含亮氨酸的胶质瘤失活蛋白1和接触蛋白相关蛋白2),并在神经元突触表面及其他部位表达。这些疾病通过降低抗体水平的免疫疗法是可逆的。虽然罕见,但在临床实践中对这些疾病的识别使得中枢神经系统自身免疫性疾病成为许多临床表现鉴别诊断中的一个考虑因素。关于这些疾病的病因、抗体作用的机制、抗体攻击后神经元和神经胶质细胞的变化以及确保良好恢复可能需要的代偿性变化,仍有许多需要了解的地方。