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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...

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Autores principales: Wang, Wanyu, Liu, Lingchun, Zhang, Mingzhi, Yang, Ruihan, Liu, Da, Yang, Shunyu, Meng, Qiang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
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.
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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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