{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T23:42:29Z","timestamp":1792798949099,"version":"4.5.0"},"reference-count":23,"publisher":"Georg Thieme Verlag KG","issue":"11","license":[{"start":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T00:00:00Z","timestamp":1792713600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arq Neuropsiquiatr"],"published-print":{"date-parts":[[2026,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Pan-neurofascinopathies represent a distinct subset of inflammatory neuropathies characterized by autoantibodies that target multiple neurofascin (NF) isoforms, which are essential for nodal and paranodal architecture in peripheral nerves. These antibodies are associated with a broad clinical spectrum, often mimicking Guillain-Barr\u00e9 syndrome (GBS) or chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and exhibit significant variability depending on the immunoglobulin G (IgG) subclass involved. The IgG1 and 3 subclasses correlate with acute, fulminant presentations and complement activation, whereas IgG4-mediated cases are typically indolent but resistant to first-line therapies. Although primarily restricted to the peripheral nervous system, pan-neurofascinopathies may also mimic central demyelinating diseases and present with systemic features such as nephrotic syndrome. These atypical manifestations highlight the expanding clinical spectrum of the disease. Early identification of anti-pan-NF antibodies and their respective IgG subclasses is crucial to guide diagnosis and management. Timely recognition enables implementation of aggressive B cell depletion-based treatment strategies, which are essential to avoid long-term neurological sequelae in affected patients.<\/jats:p>","DOI":"10.1055\/s-0046-1829127","type":"journal-article","created":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T23:15:21Z","timestamp":1792797321000},"page":"001-007","source":"Crossref","is-referenced-by-count":0,"title":["Pan-neurofascinopathies: a subgroup that mimic other neuroimmune-mediated diseases"],"prefix":"10.1055","volume":"84","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9127-7886","authenticated-orcid":false,"given":"Marcus Vin\u00edcius Magno","family":"Gon\u00e7alves","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo, Faculdade de Medicina, Programa de Neuroci\u00eancias, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1485-3771","authenticated-orcid":false,"given":"Pedro Jos\u00e9","family":"Tomaselli","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo, Faculdade de Medicina, Departamento de Neuroci\u00eancias, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1390-619X","authenticated-orcid":false,"given":"Trajano Aguiar Pires","family":"Gon\u00e7alves","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo, Faculdade de Medicina, Departamento de Neuroci\u00eancias, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4325-3414","authenticated-orcid":false,"given":"Rodrigo Siqueira Soares","family":"Frezatti","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo, Faculdade de Medicina, Departamento de Neuroci\u00eancias, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4589-2749","authenticated-orcid":false,"given":"Wilson Marques","family":"Junior","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo, Faculdade de Medicina, Departamento de Neuroci\u00eancias, Brazil"},{"name":"Universidade de S\u00e3o Paulo, Instituto Nacional de Ci\u00eancia e Tecnologia Translacional em Medicina, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2026,10,23]]},"reference":[{"issue":"10","key":"ref1","doi-asserted-by":"publisher","first-page":"1928","DOI":"10.1016\/j.clinph.2013.03.025","article-title":"Nodo-paranodopathy: beyond the demyelinating and axonal classification in anti-ganglioside antibody-mediated neuropathies","volume":"124","author":"A Uncini","year":"2013","journal-title":"Clin Neurophysiol"},{"issue":"01","key":"ref2","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1136\/jnnp-2021-326889","article-title":"New classification of autoimmune neuropathies based on target antigens and involved domains of myelinated fibres","volume":"93","author":"A Uncini","year":"2022","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"05","key":"ref3","doi-asserted-by":"publisher","first-page":"841","DOI":"10.1016\/0092-8674(87)90107-3","article-title":"Neurofascin: a novel chick cell-surface glycoprotein involved in neurite-neurite interactions","volume":"51","author":"F G Rathjen","year":"1987","journal-title":"Cell"},{"issue":"05","key":"ref4","doi-asserted-by":"publisher","first-page":"394","DOI":"10.1097\/WCO.0000000000001195","article-title":"Pan-Neurofascin autoimmune nodopathy - a life-threatening, but reversible neuropathy","volume":"36","author":"L Appeltshauser","year":"2023","journal-title":"Curr Opin Neurol"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"520","DOI":"10.3389\/fimmu.2014.00520","article-title":"IgG subclasses and allotypes: from structure to effector functions","volume":"5","author":"G Vidarsson","year":"2014","journal-title":"Front Immunol"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"779385","DOI":"10.3389\/fnmol.2021.779385","article-title":"Impact of Neurofascin on Chronic Inflammatory Demyelinating Polyneuropathy via Changing the Node of Ranvier Function: A Review","volume":"14","author":"Y Gao","year":"2021","journal-title":"Front Mol Neurosci"},{"issue":"11","key":"ref7","doi-asserted-by":"publisher","first-page":"1186","DOI":"10.1136\/jnnp-2014-310097","article-title":"Nodopathies of the peripheral nerve: an emerging concept","volume":"86","author":"A Uncini","year":"2015","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"01","key":"ref8","doi-asserted-by":"publisher","first-page":"e200365","DOI":"10.1212\/NXI.0000000000200365","article-title":"The Discovery of Autoimmune Nodopathies and the Impact of IgG4 Antibodies in Autoimmune Neurology","volume":"12","author":"L Querol","year":"2025","journal-title":"Neurol Neuroimmunol Neuroinflamm"},{"issue":"10","key":"ref9","doi-asserted-by":"publisher","first-page":"1089","DOI":"10.1136\/jnnp-2021-326343","article-title":"IgG1 pan-neurofascin antibodies identify a severe yet treatable neuropathy with a high mortality","volume":"92","author":"J Fehmi","year":"2021","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"11","key":"ref10","doi-asserted-by":"publisher","first-page":"1587","DOI":"10.3390\/brainsci12111587","article-title":"Anti-NF155\/NF186 IgG4 Antibody Positive Autoimmune Nodopathy","volume":"12","author":"L Wang","year":"2022","journal-title":"Brain Sci"},{"issue":"03","key":"ref11","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1111\/jns.12455","article-title":"European Academy of Neurology\/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision","volume":"26","author":"P YK Van den Bergh","year":"2021","journal-title":"J Peripher Nerv Syst"},{"issue":"03","key":"ref12","doi-asserted-by":"publisher","first-page":"453","DOI":"10.1177\/13524585211028126","article-title":"The clinical features of combined central and peripheral demyelination and antibodies against the node of Ranvier","volume":"28","author":"X Hou","year":"2022","journal-title":"Mult Scler"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"182","DOI":"10.1016\/j.jns.2016.02.022","article-title":"Combined central and peripheral demyelination: Clinical features, diagnostic findings, and treatment","volume":"363","author":"A Cortese","year":"2016","journal-title":"J Neurol Sci"},{"issue":"05","key":"ref14","doi-asserted-by":"publisher","first-page":"422","DOI":"10.1136\/pn-2024-004114","article-title":"Combined central and peripheral demyelination in two siblings, immune mediated or genetic?","volume":"24","author":"K Moodley","year":"2024","journal-title":"Pract Neurol"},{"issue":"08","key":"ref15","doi-asserted-by":"publisher","first-page":"714","DOI":"10.1212\/WNL.0b013e3182a1aa9c","article-title":"Anti-neurofascin antibody in patients with combined central and peripheral demyelination","volume":"81","author":"N Kawamura","year":"2013","journal-title":"Neurology"},{"issue":"01","key":"ref16","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1038\/ki.2012.321","article-title":"Neuronal proteins are novel components of podocyte major processes and their expression in glomerular crescents supports their role in crescent formation","volume":"83","author":"L Sistani","year":"2013","journal-title":"Kidney Int"},{"issue":"01","key":"ref17","doi-asserted-by":"publisher","first-page":"E1","DOI":"10.1002\/mus.26493","article-title":"Neurofascin-140 and -155 antibodies in an atypical case of poems syndrome","volume":"60","author":"T Rempe","year":"2019","journal-title":"Muscle Nerve"},{"issue":"06","key":"ref18","doi-asserted-by":"publisher","first-page":"1847","DOI":"10.1111\/ene.15266","article-title":"Daratumumab for treatment-refractory antibody-mediated diseases in neurology","volume":"29","author":"F Scheibe","year":"2022","journal-title":"Eur J Neurol"},{"issue":"04","key":"ref19","doi-asserted-by":"publisher","first-page":"475","DOI":"10.1111\/jns.12465","article-title":"Report of a fulminant anti-pan-neurofascin-associated neuropathy responsive to rituximab and bortezomib","volume":"26","author":"M Fels","year":"2021","journal-title":"J Peripher Nerv Syst"},{"issue":"04","key":"ref20","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1055\/s-0045-1805073","article-title":"Immune-mediated insights into clinical and specific autoantibodies in acute and chronic immune-mediated nodo-paranodopathies","volume":"83","author":"M VM Gon\u00e7alves","year":"2025","journal-title":"Arq Neuropsiquiatr"},{"issue":"01","key":"ref21","doi-asserted-by":"publisher","first-page":"6","DOI":"10.1038\/s41409-024-02429-6","article-title":"CAR-T cell therapy embarks on autoimmune disease","volume":"60","author":"A Rampotas","year":"2025","journal-title":"Bone Marrow Transplant"},{"key":"ref22","doi-asserted-by":"publisher","first-page":"1461102","DOI":"10.3389\/fimmu.2024.1461102","article-title":"CAR immunotherapy in autoimmune diseases: promises and challenges","volume":"15","author":"J Yu","year":"2024","journal-title":"Front Immunol"},{"issue":"05","key":"ref23","doi-asserted-by":"publisher","first-page":"1932","DOI":"10.1093\/brain\/awac418","article-title":"Anti-pan-neurofascin antibodies induce subclass-related complement activation and nodo-paranodal damage","volume":"146","author":"L Appeltshauser","year":"2023","journal-title":"Brain"}],"container-title":["Arquivos de Neuro-Psiquiatria"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/s-0046-1829127.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T23:15:24Z","timestamp":1792797324000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/s-0046-1829127"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,10,23]]},"references-count":23,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2026,8,13]]},"published-print":{"date-parts":[[2026,11]]}},"alternative-id":["s00461829127"],"URL":"https:\/\/doi.org\/10.1055\/s-0046-1829127","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0004-282X","1678-4227"],"issn-type":[{"value":"0004-282X","type":"print"},{"value":"1678-4227","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,10,23]]}}}