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Case report: Autoimmune nodopathy with concurrent serum and CSF IgG4 anti-neurofascin 155 antibodies
OBJECTIVE: To report a case of autoimmune nodopathy (AN) with concurrent serum and CSF immunoglobulin (Ig)G4 anti-neurofascin 155 (NF155) and anti-GD1b antibodies. METHODS: A 20-year-old male presented distal weakness of the 4 limbs, hypoesthesia, absent tendon reflexes and sensory ataxia. Nerve con...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2022
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9561397/ https://www.ncbi.nlm.nih.gov/pubmed/36248836 http://dx.doi.org/10.3389/fimmu.2022.1028282 |
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author | Wang, Wanyu Liu, Lingchun Zhang, Mingzhi Yang, Ruihan Liu, Da Yang, Shunyu Meng, Qiang |
author_facet | Wang, Wanyu Liu, Lingchun Zhang, Mingzhi Yang, Ruihan Liu, Da Yang, Shunyu Meng, Qiang |
author_sort | Wang, Wanyu |
collection | PubMed |
description | OBJECTIVE: To report a case of autoimmune nodopathy (AN) with concurrent serum and CSF immunoglobulin (Ig)G4 anti-neurofascin 155 (NF155) and anti-GD1b antibodies. METHODS: A 20-year-old male presented distal weakness of the 4 limbs, hypoesthesia, absent tendon reflexes and sensory ataxia. Nerve conduction studies (NCS), MRI, and autoantibody tests were performed. RESULTS: NCS revealed a diffuse demyelinating neuropathy in the peripheral nerve with motor and sensory involvement. MRI of the cervical and lumbar plexus showed diffuse enlargement. IgG4 anti-NF155 antibodies in both serum and CSF and IgG anti-GD1b antibodies in serum were positive. After treatment with IVIg, rituximab, and plasma exchange, the titer of the patient’s anti-NF155 antibodies decreased, but symptoms did not significantly improve. DISCUSSION: This patient presented a typical clinical feature of AN with serum and CSF anti-NF155 antibodies and serum anti-GD1b antibodies coexistent but poor response to IVIg, rituximab and plasma exchange. Early detection of antibodies may be helpful in both diagnosis and therapy of the disease. And prospective studies are necessary to demonstrate the potential role of anti-NF155 antibodies in CSF and help further understand this complex and heterogeneous disease. |
format | Online Article Text |
id | pubmed-9561397 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-95613972022-10-15 Case report: Autoimmune nodopathy with concurrent serum and CSF IgG4 anti-neurofascin 155 antibodies Wang, Wanyu Liu, Lingchun Zhang, Mingzhi Yang, Ruihan Liu, Da Yang, Shunyu Meng, Qiang Front Immunol Immunology OBJECTIVE: To report a case of autoimmune nodopathy (AN) with concurrent serum and CSF immunoglobulin (Ig)G4 anti-neurofascin 155 (NF155) and anti-GD1b antibodies. METHODS: A 20-year-old male presented distal weakness of the 4 limbs, hypoesthesia, absent tendon reflexes and sensory ataxia. Nerve conduction studies (NCS), MRI, and autoantibody tests were performed. RESULTS: NCS revealed a diffuse demyelinating neuropathy in the peripheral nerve with motor and sensory involvement. MRI of the cervical and lumbar plexus showed diffuse enlargement. IgG4 anti-NF155 antibodies in both serum and CSF and IgG anti-GD1b antibodies in serum were positive. After treatment with IVIg, rituximab, and plasma exchange, the titer of the patient’s anti-NF155 antibodies decreased, but symptoms did not significantly improve. DISCUSSION: This patient presented a typical clinical feature of AN with serum and CSF anti-NF155 antibodies and serum anti-GD1b antibodies coexistent but poor response to IVIg, rituximab and plasma exchange. Early detection of antibodies may be helpful in both diagnosis and therapy of the disease. And prospective studies are necessary to demonstrate the potential role of anti-NF155 antibodies in CSF and help further understand this complex and heterogeneous disease. Frontiers Media S.A. 2022-09-30 /pmc/articles/PMC9561397/ /pubmed/36248836 http://dx.doi.org/10.3389/fimmu.2022.1028282 Text en Copyright © 2022 Wang, Liu, Zhang, Yang, Liu, Yang and Meng https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Immunology Wang, Wanyu Liu, Lingchun Zhang, Mingzhi Yang, Ruihan Liu, Da Yang, Shunyu Meng, Qiang Case report: Autoimmune nodopathy with concurrent serum and CSF IgG4 anti-neurofascin 155 antibodies |
title | Case report: Autoimmune nodopathy with concurrent serum and CSF IgG4 anti-neurofascin 155 antibodies |
title_full | Case report: Autoimmune nodopathy with concurrent serum and CSF IgG4 anti-neurofascin 155 antibodies |
title_fullStr | Case report: Autoimmune nodopathy with concurrent serum and CSF IgG4 anti-neurofascin 155 antibodies |
title_full_unstemmed | Case report: Autoimmune nodopathy with concurrent serum and CSF IgG4 anti-neurofascin 155 antibodies |
title_short | Case report: Autoimmune nodopathy with concurrent serum and CSF IgG4 anti-neurofascin 155 antibodies |
title_sort | case report: autoimmune nodopathy with concurrent serum and csf igg4 anti-neurofascin 155 antibodies |
topic | Immunology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9561397/ https://www.ncbi.nlm.nih.gov/pubmed/36248836 http://dx.doi.org/10.3389/fimmu.2022.1028282 |
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