Department of Neurology, Medical Faculty, Heinrich Heine University, Düsseldorf, Germany.
Neuroinflammation Laboratory, Brain and Mind Centre, University of Sydney, Sydney, Australia.
Nat Rev Dis Primers. 2018 Oct 11;4(1):31. doi: 10.1038/s41572-018-0027-2.
Since the discovery of an acute monophasic paralysis, later coined Guillain-Barré syndrome, almost 100 years ago, and the discovery of chronic, steroid-responsive polyneuropathy 50 years ago, the spectrum of immune-mediated polyneuropathies has broadened, with various subtypes continuing to be identified, including chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN). In general, these disorders are speculated to be caused by autoimmunity to proteins located at the node of Ranvier or components of myelin of peripheral nerves, although disease-associated autoantibodies have not been identified for all disorders. Owing to the numerous subtypes of the immune-mediated neuropathies, making the right diagnosis in daily clinical practice is complicated. Moreover, treating these disorders, particularly their chronic variants, such as CIDP and MMN, poses a challenge. In general, management of these disorders includes immunotherapies, such as corticosteroids, intravenous immunoglobulin or plasma exchange. Improvements in clinical criteria and the emergence of more disease-specific immunotherapies should broaden the therapeutic options for these disabling diseases.
自近 100 年前发现急性单相麻痹（后称为格林-巴利综合征）和 50 年前发现慢性、类固醇反应性多发性神经病以来，免疫介导性多发性神经病的范围已经扩大，各种亚型不断被发现，包括慢性炎症性脱髓鞘性多发性神经病（CIDP）和多灶性运动神经病（MMN）。一般来说，这些疾病被推测是由位于Ranvier 结或周围神经髓鞘成分的自身抗体引起的，尽管并非所有疾病都能识别出与疾病相关的自身抗体。由于免疫介导性神经病的众多亚型，在日常临床实践中做出正确的诊断很复杂。此外，治疗这些疾病，特别是 CIDP 和 MMN 等慢性变异型，也具有挑战性。一般来说，这些疾病的治疗包括免疫疗法，如皮质类固醇、静脉注射免疫球蛋白或血浆置换。临床标准的改进和更多疾病特异性免疫疗法的出现，应该会为这些致残性疾病提供更多的治疗选择。
