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Autoimmune nodopathies: treatable neuropathies beyond traditional classifications
  1. Luis Querol1,2
  1. 1 Neuromuscular Diseases Unit, Neurology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain
  2. 2 Centro de Investigación Biomédica en Red para Enfermedades Raras (CIBERER), Madrid, Spain
  1. Correspondence to Dr Luis Querol, Neuromuscular Diseases Unit, Neurology, Hospital de la Santa Creu i Sant Pau, Barcelona 08041, Spain; lquerol{at}santpau.cat

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Precise characterisation of the target antigens and autoantibody isotypes improves patient care in inflammatory neuropathies

Antibodies targeting all neurofascin isoforms (pan-neurofascin) have been associated with variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) that include ataxic presentations, cranial nerve involvement, respiratory failure and association with other autoimmune disorders .1 2 Fehmi et al describe a severe, yet treatable, neuropathy associated with anti-pan-neurofascin antibodies of the IgG1 isotype.3

Nodo-paranodal antibodies have been described in neuropathies fulfilling CIDP diagnostic criteria. However, a significant proportion of patients harbouring any nodo-paranodal autoantibody (contactin-1, contactin-associated protein-1, neurofascin-155 or pan-neurofascin) presents as rapidly progressive, aggressive (including the need of intensive care unit admission and need of mechanical ventilation) neuropathies, often leading to an initial diagnosis of Guillain-Barré syndrome. Fehmi et al describe that anti-pan-neurofascin IgG1 antibodies associate with an …

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Footnotes

  • Contributors LAQ has created and edited this manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests LAQ’s main field of research is autoantibodies in inflammatory neuropathies. His institution provides routine diagnostic testing for nodo-paranodal antibodies.

  • Provenance and peer review Commissioned; internally peer reviewed.

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