Within the last decade, antibodies targeting the node and paranode of myelinated peripheral nerves have been increasingly identified in patients with acquired immune-mediated neuropathies, commonly termed ‘nodo-paranodopathies’. Crucially, these patients often present with additional clinical features not usually seen with the most common immune-mediated neuropathies, Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy, and respond poorly to conventionally used immunomodulatory therapies. Emerging evidence that these are pathologically distinct diseases has further prompted the use of more targeted treatment, such as the B cell depleting monoclonal antibody rituximab, which has been reported to significantly improve functional outcomes in this subset of patients. We provide an overview of the emerging clinical and serological phenotypes in patients with specific nodal/paranodal antibodies, the practicalities of antibody testing and current evidence supporting the use of non-standard therapies.

中文翻译：

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结节和节旁抗体相关神经病


在过去的十年中，越来越多地在患有获得性免疫介导的神经病（通常称为“结节性偏神经病”）的患者中发现了针对有髓周围神经的节点和旁节点的抗体。至关重要的是，这些患者通常会出现最常见的免疫介导的神经病、吉兰-巴利综合征和慢性炎症性脱髓鞘性多发性神经病中不常见的额外临床特征，并且对常规使用的免疫调节疗法反应不佳。越来越多的证据表明这些是病理上不同的疾病，进一步促使使用更有针对性的治疗，例如 B 细胞消耗单克隆抗体利妥昔单抗，据报道它可以显着改善这部分患者的功能结果。我们概述了具有特定淋巴结/淋巴结旁抗体的患者的新出现的临床和血清学表型、抗体检测的实用性以及支持使用非标准疗法的当前证据。