[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"detail-sidebar-cat-0-en-105":3,"doc-seo-435932-105":59,"doc-detail-435932-en":130},{"code":4,"msg":5,"data":6},0,"success",[7,13,18,23,28,33,38,43,48,51,55],{"id":8,"doc_module":4,"doc_module_name":9,"category_name":10,"show_sort_weight":11,"slug":12},1,"Document","Story & Novel",90,"story-novel",{"id":14,"doc_module":4,"doc_module_name":9,"category_name":15,"show_sort_weight":16,"slug":17},2,"Literature",80,"literature",{"id":19,"doc_module":4,"doc_module_name":9,"category_name":20,"show_sort_weight":21,"slug":22},4,"Exam",70,"exam",{"id":24,"doc_module":4,"doc_module_name":9,"category_name":25,"show_sort_weight":26,"slug":27},5,"Comic",60,"comic",{"id":29,"doc_module":4,"doc_module_name":9,"category_name":30,"show_sort_weight":31,"slug":32},6,"Technology",50,"technology",{"id":34,"doc_module":4,"doc_module_name":9,"category_name":35,"show_sort_weight":36,"slug":37},7,"Healthcare",40,"healthcare",{"id":39,"doc_module":4,"doc_module_name":9,"category_name":40,"show_sort_weight":41,"slug":42},8,"Research & Report",30,"research-report",{"id":44,"doc_module":4,"doc_module_name":9,"category_name":45,"show_sort_weight":46,"slug":47},9,"Religion & Spirituality",20,"religion-spirituality",{"id":46,"doc_module":4,"doc_module_name":9,"category_name":49,"show_sort_weight":46,"slug":50},"World Cup","world-cup",{"id":52,"doc_module":4,"doc_module_name":9,"category_name":53,"show_sort_weight":52,"slug":54},10,"Lifestyle","lifestyle",{"id":56,"doc_module":4,"doc_module_name":9,"category_name":57,"show_sort_weight":24,"slug":58},19,"General","general",{"code":4,"msg":60,"data":61},"ok",{"site_id":62,"language":63,"slug":64,"title":65,"keywords":66,"description":67,"schema_data":68,"social_meta":123,"head_meta":125,"extra_data":127,"updated_unix":129},105,"en","anti-pan-neurofascin-igg3-insights-about-an-emerging-autoimmune-nodoparanodopathy","Anti-pan-neurofascin IgG3: insights about an emerging autoimmune nodoparanodopathy","","Neurofascin family proteins drive a spectrum of autoimmune nodoparanodopathies as CIDP subtypes emerge with distinct mechanisms. Among them, anti-pan-neurofascin IgG3 shows a particularly severe, diagnostically challenging phenotype, often with fulminant progression and high morbidity and mortality. This review summarizes seven reported cases and proposes a pathophysiology centered on Ranvier node destruction via hyperactivated membrane attack complex (MAC) formation. It also evaluates emerging evidence for eculizumab and discusses standardized antibody assays, serology, and neurophysiology to improve diagnosis.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/anti-pan-neurofascin-igg3-insights-about-an-emerging-autoimmune-nodoparanodopathy/435932/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/anti-pan-neurofascin-igg3-insights-about-an-emerging-autoimmune-nodoparanodopathy/435932.png","ImageObject",300,407,{"name":92,"@type":93},"Finn","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-29","2026-09-29",true,{"@type":102,"interactionType":103,"userInteractionCount":14},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"What is anti-pan-neurofascin IgG3 and why is it clinically important?","Question",{"text":112,"@type":113},"Anti-pan-neurofascin IgG3 is a subtype of antibody-mediated autoimmune nodoparanodopathy. It is highlighted for a severe, diagnostically challenging course with high morbidity and mortality.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"How does the review explain the proposed mechanism of disease in anti-pan-neurofascin IgG3?",{"text":117,"@type":113},"The review proposes destruction of the node of Ranvier through hyperactivation of membrane attack complex (MAC) formation.",{"name":119,"@type":110,"acceptedAnswer":120},"What diagnostic and treatment approaches does the review emphasize?",{"text":121,"@type":113},"It discusses using standardized antibody assays, serological analyses, and neurophysiological studies to improve diagnostic accuracy, and it examines emerging evidence for eculizumab alongside other treatment strategies.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},435932,1792235198,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":14,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":39,"language":139,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":67,"update_tm":143,"read_time":46,"preview_page_count":4},34359740700684,"https://ap-avatar.wpscdn.com/avatar/1f400023980c374ae676?_k=1777273430885731487","Article published online: 2025-10-15  \nReview Article 1  \nAnti-pan-neurofascin IgG3: insights about an emerging autoimmune nodoparanodopathy  \nGabriel Erzinger 1 Mayra Emi Guinoza Inushi 1 Laura Fiuza Parolin 1 Gabriel de Deus Vieira 1 Marcus Vinícius Magno Gonçalves 1  \n1 Universidade da Região de Joinville, Departamento de Medicina, Joinville SC, Brazil.  \nArq. Neuro-Psiquiatr. 2025;83(9):s00451812035 .  \nAddress for correspondence Gabriel Erzinger (email: [gabierzinger@gmail.com](gabierzinger@gmail.com))  \nAbstract  \nKeywords  \n► Polyneuropathies  \n► Autoantibodies  \n► Immunoglobulin G  \nNeurofascin constitutes a family of cell-surface proteins identified more than 4 decades ago, produced through alternative RNA splicing, with various isoforms expressed in neural tissues. With the emergence of chronic inflammatory demyelinating polyneuropathy (CIDP) subtypes characterized by distinct pathological mechanisms, antineurofascin antibody-mediated neuropathies have gained attention and are now categorized as autoimmune nodoparanodopathies. Among these, the anti-pan-neurofascin immunoglobulin G3 (IgG3) subtype presents a particularly severe and diagnostically-challenging phenotype, marked by a fulminant clinical course, diverse symptomatology, and high rates of morbidity and mortality. Despite its clinical relevance, to date, no comprehensive review has focused specifically on this manifestation, highlighting a significant gap in the literature. To address this, we herein review the seven reported cases and explore the proposed pathophysiological mechanism involving the destruction of the node of Ranvier via hyperactivation of membrane attack complex (MAC) formation. Additionally, we examine emerging evidence supporting the use of eculizumab as a potential therapeutic option, alongside other treatment strategies. Finally, we discuss the role of standardized antibody assays, serological analyses, and neurophysiological studies in improving diagnostic accuracy.  \nINTRODUCTION  \nIn 2013, Uncini et al.1 proposed a new classification for dysfunctions of the node of Ranvier, termed nodoparanodopathy, initially described in antiganglioside antibody-mediated neuropathies. This classification differs from the traditional demyelinating and axonal classifications, focusing on the specific site of action, such as the node, paranode, juxtaparanode, or internode regions. The previous classifi cation divides polyneuropathies into two groups: the first affects the myelin/Schwann cells, and the second affects the  \nreceived  \nJanuary 7, 2025  \nreceived in its final form May 23, 2025  \naccepted July 27, 2025  \nDOI [https://doi.org/](https://doi.org/)[ ](https://doi.org/)10.1055/s-0045-1812035 . ISSN 0004-282X.  \nEditor-in-Chief: Ayrton Roberto Massaro.  \nAssociate Editor: Wilson Marques Jr.  \naxon, which often leads to imprecise diagnoses in some electrophysiological findings, particularly in Guillain-Barré syndrome (GBS) subtypes. This paradigm shift is significant as it provides a more precise correlation between clinical presentation and underlying pathophysiological mechanisms, challenging the notion that the nodal region is the sole pathological target and the misconception of a universally-poor prognosis in such diseases.1 Furthemore, in those [regions. an](regions. an) antibody target of relevance to the clinical practice is the neurofascin (NF) family of proteins, first described in 1987 in chicks. These neural surface proteins  \n© 2025 . The Author(s) .  \nThis is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit ([https://creativecommons.org/licenses/by/4.0/](https://creativecommons.org/licenses/by/4.0/))  \nThieme Revinter Publicações Ltda. , Rua Rego Freitas, 175, loja 1, República, São Paulo, SP, CEP 01220-010, Brazil  \n2 Anti-pan-neurofascin IgG3: a review Erzinger et al.  \nhave several i","cbCaitK7BhkfxCKh","https://ap.wps.com/l/cbCaitK7BhkfxCKh","pdf",3055997,"English","# Abstract\n# Keywords\n# Introduction\n## Node of Ranvier dysfunction classification\n## Neurofascin isoforms and antibody targets\n## Autoimmune nodopathies and treatment guidelines","[{\"question\":\"What is anti-pan-neurofascin IgG3 and why is it clinically important?\",\"answer\":\"Anti-pan-neurofascin IgG3 is a subtype of antibody-mediated autoimmune nodoparanodopathy. It is highlighted for a severe, diagnostically challenging course with high morbidity and mortality.\"},{\"question\":\"How does the review explain the proposed mechanism of disease in anti-pan-neurofascin IgG3?\",\"answer\":\"The review proposes destruction of the node of Ranvier through hyperactivation of membrane attack complex (MAC) formation.\"},{\"question\":\"What diagnostic and treatment approaches does the review emphasize?\",\"answer\":\"It discusses using standardized antibody assays, serological analyses, and neurophysiological studies to improve diagnostic accuracy, and it examines emerging evidence for eculizumab alongside other treatment strategies.\"}]","Anti-pan-neurofascin IgG3: insights about an emerging autoimmune nodoparanodopathy | PDF",1790676004]