• Jean-Michel Vallat, Laurent Magy, Philippe Corcia, Jean-Marc Boulesteix, Antonino Uncini, and Stéphane Mathis.
• From the Department of Neurology, National Reference Center for 'Rare Peripheral Neuropathies', University Hospital, Limoges, France.
• J. Neuropathol. Exp. Neurol. 2020 Mar 1; 79 (3): 247-255.

AbstractWhatever 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 "nodo-paranodopathy" was first applied to some "axonal Guillain-Barré syndrome" subtypes, then extended to cases classified as chronic inflammatory demyelinating polyradiculoneuropathy 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. Electron microscopy of longitudinal sections of nerve biopsies is useful to visualize and authenticate the characteristic lesions of paranodes/nodes. However, it should be borne in mind that identical ultrastructural aspects are seen in other types of polyneuropathies: Genetic, experimental, and in a few polyneuropathies for which there is no obvious etiology. Ultrastructural nerve studies confirm the initial involvement of nodes/paranodes in various types of acquired and genetic neuropathies. For some of them, the antibodies or the proteins involved by mutations are clearly identified such as Caspr-1, Contactin-1, NFasc155, and NFasc186; other unidentified proteins are likely to be involved as well.© 2019 American Association of Neuropathologists, Inc. All rights reserved.

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