KMID : 1022820220130020023
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Journal of Multiple Sclerosis and Neuroimmunology 2022 Volume.13 No. 2 p.23 ~ p.29
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Autoimmune Nodopathies
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Lyou Hyun-Ji
Shin Ha-Young
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Abstract
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utoantibodies against proteins at the node of Ranvier such as neurofascin-155, contactin-1, contactin-associated protein-1, and neurofascin-140/186 have been discovered in a proportion of patients with peripheral neuropathy fulfilling the previous diagnostic criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) (for example, European Federation of Neurological Societies and the Peripheral Nerve Society [EFNS/PNS] 2010 criteria). Although peripheral neuropathies with these autoantibodies can meet the criteria, they show distinct clinical characteristics, such as poor response to intravenous immunoglobulin and/ or corticosteroids and no macrophage-mediated demyelination or overt inflammation, which are not typical of CIDP. For these reasons, a new concept of ¡°autoimmune nodopathy¡± has been proposed and distinguished from CIDP. This review briefly summarized the structure of the node of Ranvier and autoimmune nodopathies associated with autoantibodies against neurofascin-155, contactin-1, contactin-associated protein-1, and neurofascin-140/186.
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KEYWORD
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Chronic inflammatory demyelinating polyradiculoneuropathy, Ranvier¡¯s nodes, Autoantibodies
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