Pan-neurofascinopathies: a subgroup that mimic other neuroimmune-mediated diseases
- 1Universidade de São Paulo, Faculdade de Medicina, Programa de Neurociências
- 2Universidade de São Paulo, Faculdade de Medicina, Departamento de Neurociências
Abstract
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-Barré 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.
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BibTeX
@article{goncalves2026panneurofascinopathies,
title = {{Pan-neurofascinopathies: a subgroup that mimic other neuroimmune-mediated diseases}},
author = {Marcus Vinícius Magno Gonçalves and Pedro José Tomaselli and Trajano Aguiar Pires Gonçalves and Rodrigo Siqueira Soares Frezatti and Wilson Marques Junior},
journal = {Arquivos de neuro-psiquiatria},
year = {2026},
month = oct,
doi = {10.1055/s-0046-1829127},
url = {https://europepmc.org/article/PMC/PMC13694134},
note = {CC BY},
}
RIS
TY - JOUR
TI - Pan-neurofascinopathies: a subgroup that mimic other neuroimmune-mediated diseases
AU - Marcus Vinícius Magno Gonçalves
AU - Pedro José Tomaselli
AU - Trajano Aguiar Pires Gonçalves
AU - Rodrigo Siqueira Soares Frezatti
AU - Wilson Marques Junior
PY - 2026
DA - 2026/10/23
JO - Arquivos de neuro-psiquiatria
DO - 10.1055/s-0046-1829127
UR - https://europepmc.org/article/PMC/PMC13694134
AB - 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-Barré 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.
SN - 0004-282X
SN - 1678-4227
ER -