Back to Search
Start Over
IgG1pan-neurofascin antibodies identify a severe yet treatable neuropathy with a high mortality
- Source :
- Journal of Neurology, Neurosurgery, & Psychiatry (JNNP); 2021, Vol. 92 Issue: 10 p1089-1095, 7p
- Publication Year :
- 2021
-
Abstract
- ObjectivesWe aimed to define the clinical and serological characteristics of pan-neurofascin antibody-positive patients.MethodsWe tested serum from patients with suspected immune-mediated neuropathies for antibodies directed against nodal/paranodal protein antigens using a live cell-based assay and solid-phase platform. The clinical and serological characteristics of antibody-positive and seronegative patients were then compared. Sera positive for pan-neurofascin were also tested against live myelinated human stem cell-derived sensory neurons for antibody binding.ResultsEight patients with IgG1-subclass antibodies directed against both isoforms of the nodal/paranodal cell adhesion molecule neurofascin were identified. All developed rapidly progressive tetraplegia. Cranial nerve deficits (100% vs 26%), autonomic dysfunction (75% vs 13%) and respiratory involvement (88% vs 14%) were more common than in seronegative patients. Four patients died despite treatment with one or more modalities of standard immunotherapy (intravenous immunoglobulin, steroids and/or plasmapheresis), whereas the four patients who later went on to receive the B cell-depleting therapy rituximab then began to show progressive functional improvements within weeks, became seronegative and ultimately became functionally independent.ConclusionsIgG1pan-neurofascin antibodies define a very severe autoimmune neuropathy. We urgently recommend trials of targeted immunotherapy for this serologically classified patient group.
Details
- Language :
- English
- ISSN :
- 00223050 and 1468330X
- Volume :
- 92
- Issue :
- 10
- Database :
- Supplemental Index
- Journal :
- Journal of Neurology, Neurosurgery, & Psychiatry (JNNP)
- Publication Type :
- Periodical
- Accession number :
- ejs57825786
- Full Text :
- https://doi.org/10.1136/jnnp-2021-326343