التفاصيل البيبلوغرافية
| العنوان: |
IgG1 pan-neurofascin antibodies identify a severe yet treatable neuropathy with a high mortality |
| المؤلفون: |
Janev Fehmi, Alexander J Davies, Jon Walters, Timothy Lavin, Ryan Keh, Alexander M Rossor, Tudor Munteanu, Norman Delanty, Rhys Roberts, Dirk Bäumer, Graham Lennox, Simon Rinaldi |
| سنة النشر: |
2023 |
| مصطلحات موضوعية: |
Immunology, Central nervous system, Neurology and neuromuscular diseases, Peripheral Nervous System Diseases, Adrenal Cortex Hormones, Immunoglobulin G, Immunoglobulins, Intravenous, Autoantibodies, neuroimmunology, neuropathy, Autoimmune Disease, Neurodegenerative, Neurosciences, Biotechnology, Peripheral Neuropathy, Immunization, Inflammatory and immune system, IgG1 pan-neurofascin antibodies |
| الوصف: |
Objectives: We aimed to define the clinical and serological characteristics of pan-neurofascin antibody-positive patients. Methods: We 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. Results: Eight 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. Conclusions: IgG1 pan-neurofascin antibodies define a very severe autoimmune neuropathy. We urgently recommend trials of targeted immunotherapy for this serologically classified patient group. |
| نوع الوثيقة: |
article in journal/newspaper |
| اللغة: |
unknown |
| العلاقة: |
10779/rcsi.24592668.v1; https://figshare.com/articles/journal_contribution/IgG1_pan-neurofascin_antibodies_identify_a_severe_yet_treatable_neuropathy_with_a_high_mortality/24592668Test |
| الإتاحة: |
https://figshare.com/articles/journal_contribution/IgG1_pan-neurofascin_antibodies_identify_a_severe_yet_treatable_neuropathy_with_a_high_mortality/24592668Test |
| حقوق: |
CC BY 4.0 |
| رقم الانضمام: |
edsbas.D752EDBB |
| قاعدة البيانات: |
BASE |
