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Review

Antibody-Mediated Nodo- and Paranodopathies

1
Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, 1090 Vienna, Austria
2
Department of Pediatric Neurology, Children’s Hospital Datteln, University Witten/Herdecke, 45711 Datteln, Germany
*
Author to whom correspondence should be addressed.
J. Clin. Med. 2024, 13(19), 5721; https://doi.org/10.3390/jcm13195721
Submission received: 7 August 2024 / Revised: 12 September 2024 / Accepted: 22 September 2024 / Published: 25 September 2024

Abstract

The recent discovery of pathogenic antibodies targeting cell adhesion molecules of the node of Ranvier has prompted efforts to develop a new classification for a subset of antibody-mediated peripheral neuropathies. These autoimmune nodo- and paranodopathies encompass epitopes such as neurofascin 155, neurofascin 186, contactin-1, and contactin-associated protein 1, with a high likelihood of involving additional yet unidentified proteins. So far, the investigation of this subset of patients was primarily focused on adults, with only rare reports of pediatric cases. Low awareness among pediatricians and insufficient availability of appropriate diagnostic methods in many laboratories may mask a higher pediatric incidence than currently observed. Diagnosis is made by transfected cell-based assays and ELISA to characterize the specific target antigen and antibody subclass that provides insight into the pathophysiology. Clinical features often resemble those of CIDP or GBS in adults, whilst in pediatric patients, although rare, an atypical CIDP phenotype has predominantly been reported. Yet, in contrast to classical immune-mediated neuropathies, the clinical course is usually rapidly progressive, and response to classical first-line therapy often poor. Although electrophysiological signs of demyelination are observed, segmental demyelination and inflammation are not present on pathological examination. Rather, few neuropathological reports demonstrate features of axonal neuropathy without signs of true de- or remyelination. This review aims to summarize recent findings on such nodo- and paranodoneuropathies, shining light on features of these disorders in pediatric patients, a still little-explored field with only a few reports currently present.
Keywords: paranodopathy; nodopathy; neurofascin 155; neurofascin 186/140; pan-neurofascin contactin 1; Caspr1; CNTN1/Caspr1 paranodopathy; nodopathy; neurofascin 155; neurofascin 186/140; pan-neurofascin contactin 1; Caspr1; CNTN1/Caspr1

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MDPI and ACS Style

Quinot, V.; Rostasy, K.; Höftberger, R. Antibody-Mediated Nodo- and Paranodopathies. J. Clin. Med. 2024, 13, 5721. https://doi.org/10.3390/jcm13195721

AMA Style

Quinot V, Rostasy K, Höftberger R. Antibody-Mediated Nodo- and Paranodopathies. Journal of Clinical Medicine. 2024; 13(19):5721. https://doi.org/10.3390/jcm13195721

Chicago/Turabian Style

Quinot, Valérie, Kevin Rostasy, and Romana Höftberger. 2024. "Antibody-Mediated Nodo- and Paranodopathies" Journal of Clinical Medicine 13, no. 19: 5721. https://doi.org/10.3390/jcm13195721

APA Style

Quinot, V., Rostasy, K., & Höftberger, R. (2024). Antibody-Mediated Nodo- and Paranodopathies. Journal of Clinical Medicine, 13(19), 5721. https://doi.org/10.3390/jcm13195721

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