Department of Neurology, Southmead Hospital, Bristol, UK.
Department of Neurology, The Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
J Neurol Neurosurg Psychiatry. 2018 Jan;89(1):61-71. doi: 10.1136/jnnp-2016-315480. Epub 2017 Aug 17.
This review summarises recent evidence supporting the involvement of the specialised nodal and perinodal domains (the paranode and juxtaparanode) of myelinated axons in the pathology of acquired, inflammatory, peripheral neuropathies.The identification of new target antigens in the inflammatory neuropathies heralds a revolution in diagnosis, and has already begun to inform increasingly targeted and individualised therapies. Rapid progress in our basic understanding of the highly specialised nodal regions of peripheral nerves serves to strengthen the links between their unique microstructural identities, functions and pathologies. In this context, the detection of autoantibodies directed against nodal and perinodal targets is likely to be of increasing clinical importance. Antiganglioside antibodies have long been used in clinical practice as diagnostic serum biomarkers, and associate with specific clinical variants but not to the common forms of either acute or chronic demyelinating autoimmune neuropathy. It is now apparent that antibodies directed against several region-specific cell adhesion molecules, including neurofascin, contactin and contactin-associated protein, can be linked to phenotypically distinct peripheral neuropathies. Importantly, the immunological characteristics of these antibodies facilitate the prediction of treatment responsiveness.
这篇综述总结了最近的证据，支持有髓轴突的特化神经节和周围神经节域（神经节旁区和近旁节区）参与获得性炎症性周围神经病的病理过程。在炎症性神经病中识别新的靶抗原预示着诊断的革命，并且已经开始为越来越有针对性和个体化的治疗提供信息。我们对周围神经高度特化神经节区域的基础理解的快速进展有助于加强其独特的微观结构特征、功能和病理之间的联系。在这种情况下，针对神经节和神经节旁靶点的自身抗体的检测可能具有越来越重要的临床意义。抗神经节苷脂抗体在临床实践中早已被用作诊断血清生物标志物，与特定的临床亚型相关，但与急性或慢性脱髓鞘自身免疫性神经病的常见形式无关。现在很明显，针对几种区域特异性细胞黏附分子的抗体，包括神经束蛋白、接触蛋白和接触蛋白相关蛋白，可以与表型不同的周围神经病相关。重要的是，这些抗体的免疫学特征有助于预测治疗反应性。
