Case report: Anti-CNTN1 antibody-associated nodopathies disease with asymmetric onset
Anti-contactin-1 (CNTN1) IgG4 antibody-associated nodopathies is an autoimmune antibody-mediated peripheral neuropathy with a unique clinical presentation, pathophysiology, electrophysiology, and therapeutic response. The critical histopathological features are a dense lymphoplasmacytic infiltrate,...
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ftpubmed:oai:pubmedcentral.nih.gov:10035883 2023-05-15T16:01:57+02:00 Case report: Anti-CNTN1 antibody-associated nodopathies disease with asymmetric onset Li, Qian Chen, Qing Zhang, Ting Xu, Ying Kan, Yanmin Zhang, Jing 2023-03-09 http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10035883/ https://doi.org/10.3389/fneur.2023.1124540 en eng Frontiers Media S.A. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10035883/ http://dx.doi.org/10.3389/fneur.2023.1124540 Copyright © 2023 Li, Chen, Zhang, Xu, Kan and Zhang. 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. Front Neurol Neurology Text 2023 ftpubmed https://doi.org/10.3389/fneur.2023.1124540 2023-03-26T02:20:54Z Anti-contactin-1 (CNTN1) IgG4 antibody-associated nodopathies is an autoimmune antibody-mediated peripheral neuropathy with a unique clinical presentation, pathophysiology, electrophysiology, and therapeutic response. The critical histopathological features are a dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis. Here, a 62-year-old male patient presented with subacute unilateral limb onset, progressive exacerbation, marked weakness of the extremities, cranial, and autonomic nerve involvement. Neurophysiology showed slowed motor nerve conduction velocity (MCV), prolonged distal motor delay (DML), slowed sensory nerve conduction velocity (SCV), decreased sensory nerve activity potential (SNAP) amplitude, decreased amplitude of bilateral neuromotor conduction, abnormal cutaneous sympathetic response (SSR) in both lower extremities, axonal damage, prolonged F-wave latency, and discrete waves. In the initial phase, there was a response to intravenous immunoglobulin (IVIG), and corticosteroids and rituximab were also effective. After 1 year follow-up, the patient improved significantly. This article reports on a patient with nodular disease with anti-contactin-1 (CNTN1) IgG4 antibodies and reviews the literature to improve clinicians' understanding of the disease. Text DML PubMed Central (PMC) Frontiers in Neurology 14 |
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Neurology |
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Neurology Li, Qian Chen, Qing Zhang, Ting Xu, Ying Kan, Yanmin Zhang, Jing Case report: Anti-CNTN1 antibody-associated nodopathies disease with asymmetric onset |
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Neurology |
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Anti-contactin-1 (CNTN1) IgG4 antibody-associated nodopathies is an autoimmune antibody-mediated peripheral neuropathy with a unique clinical presentation, pathophysiology, electrophysiology, and therapeutic response. The critical histopathological features are a dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis. Here, a 62-year-old male patient presented with subacute unilateral limb onset, progressive exacerbation, marked weakness of the extremities, cranial, and autonomic nerve involvement. Neurophysiology showed slowed motor nerve conduction velocity (MCV), prolonged distal motor delay (DML), slowed sensory nerve conduction velocity (SCV), decreased sensory nerve activity potential (SNAP) amplitude, decreased amplitude of bilateral neuromotor conduction, abnormal cutaneous sympathetic response (SSR) in both lower extremities, axonal damage, prolonged F-wave latency, and discrete waves. In the initial phase, there was a response to intravenous immunoglobulin (IVIG), and corticosteroids and rituximab were also effective. After 1 year follow-up, the patient improved significantly. This article reports on a patient with nodular disease with anti-contactin-1 (CNTN1) IgG4 antibodies and reviews the literature to improve clinicians' understanding of the disease. |
format |
Text |
author |
Li, Qian Chen, Qing Zhang, Ting Xu, Ying Kan, Yanmin Zhang, Jing |
author_facet |
Li, Qian Chen, Qing Zhang, Ting Xu, Ying Kan, Yanmin Zhang, Jing |
author_sort |
Li, Qian |
title |
Case report: Anti-CNTN1 antibody-associated nodopathies disease with asymmetric onset |
title_short |
Case report: Anti-CNTN1 antibody-associated nodopathies disease with asymmetric onset |
title_full |
Case report: Anti-CNTN1 antibody-associated nodopathies disease with asymmetric onset |
title_fullStr |
Case report: Anti-CNTN1 antibody-associated nodopathies disease with asymmetric onset |
title_full_unstemmed |
Case report: Anti-CNTN1 antibody-associated nodopathies disease with asymmetric onset |
title_sort |
case report: anti-cntn1 antibody-associated nodopathies disease with asymmetric onset |
publisher |
Frontiers Media S.A. |
publishDate |
2023 |
url |
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10035883/ https://doi.org/10.3389/fneur.2023.1124540 |
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DML |
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DML |
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Front Neurol |
op_relation |
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10035883/ http://dx.doi.org/10.3389/fneur.2023.1124540 |
op_rights |
Copyright © 2023 Li, Chen, Zhang, Xu, Kan and Zhang. 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. |
op_doi |
https://doi.org/10.3389/fneur.2023.1124540 |
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Frontiers in Neurology |
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14 |
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