Department and Laboratory of Neurology, National Reference Center for 'Rare Peripheral Neuropathies', University Hospital of Limoges (CHU Limoges), Limoges, France.
Department of Neurology (Nerve-Muscle Unit), 'Grand Sud-Ouest' National Reference Center for Neuromuscular Disorders, ALS Center, University Hospital of Bordeaux (CHU Bordeaux), Bordeaux, France.
Brain Pathol. 2024 Mar;34(2):e13184. doi: 10.1111/bpa.13184. Epub 2023 Jun 25.
Autoimmune neuropathies are a heterogeneous group of rare and disabling diseases in which the immune system is thought to target antigens in the peripheral nervous system: they usually respond to immune therapies. Guillain-Barré syndrome is divided into several subtypes including "acute inflammatory demyelinating polyradiculoneuropathy," "acute motor axonal neuropathy," "acute motor sensory neuropathy," and other variants. Chronic forms such as chronic inflammatory demyelinating polyneuropathy (CIDP) and other subtypes and polyneuropathy associated with IgM monoclonal gammopathy; autoimmune nodopathies also belong to this group of auto-immune neuropathies. It has been shown that immunoglobulin G from the serum of about 30% of CIDP patients immunolabels nodes of Ranvier or paranodes of myelinated axons. Whatever the cause of myelin damage of the peripheral nervous system, the initial attack on myelin by a dysimmune process may begin either at the internodal area or in the paranodal and nodal regions. The term "nodoparanodopathy" was first applied to some "axonal Guillain-Barré syndrome" subtypes, then extended to cases classified as CIDP bearing IgG4 antibodies against paranodal axoglial proteins. In these cases, paranodal dissection develops in the absence of macrophage-induced demyelination. In contrast, the mechanisms of demyelination of other dysimmune neuropathies induced by macrophages are unexplained, as no antibodies have been identified in such cases. The main objective of this presentation is to show that the pathology illustrates, confirms, and may explain such mechanisms.
自身免疫性神经病是一组罕见且使人丧失能力的疾病，免疫系统被认为针对周围神经系统中的抗原：它们通常对免疫疗法有反应。格林-巴利综合征分为几个亚型，包括“急性炎症性脱髓鞘性多发性神经病”、“急性运动轴索性神经病”、“急性运动感觉神经病”和其他变体。慢性形式如慢性炎症性脱髓鞘性多发性神经病（CIDP）和其他亚型以及与 IgM 单克隆丙种球蛋白相关的多发性神经病；自身免疫性神经节病也属于这组自身免疫性神经病。已经表明，CIDP 患者血清中的免疫球蛋白 G 约有 30%免疫标记Ranvier 结或有髓轴突的旁结。无论周围神经系统髓鞘损伤的原因如何，免疫失调过程对髓鞘的初始攻击可能始于节间区或旁结和结区。“结旁神经节病”一词最初应用于一些“轴索性格林-巴利综合征”亚型，然后扩展到被归类为 CIDP 的病例，这些病例携带针对旁结轴突胶质蛋白的 IgG4 抗体。在这些情况下，在没有巨噬细胞诱导脱髓鞘的情况下发生旁结分离。相比之下，巨噬细胞诱导的其他免疫失调性神经病脱髓鞘的机制尚不清楚，因为在这种情况下没有鉴定出抗体。本次演讲的主要目的是表明病理学说明了、证实了并且可能解释了这些机制。
