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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crfrontiers:10.3389/fneur.2023.1124540 2024-06-23T07:52:23+00:00 Case report: Anti-CNTN1 antibody-associated nodopathies disease with asymmetric onset Li, Qian Chen, Qing Zhang, Ting Xu, Ying Kan, Yanmin Zhang, Jing 2023 http://dx.doi.org/10.3389/fneur.2023.1124540 https://www.frontiersin.org/articles/10.3389/fneur.2023.1124540/full unknown Frontiers Media SA https://creativecommons.org/licenses/by/4.0/ Frontiers in Neurology volume 14 ISSN 1664-2295 journal-article 2023 crfrontiers https://doi.org/10.3389/fneur.2023.1124540 2024-06-11T04:09:21Z 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. Article in Journal/Newspaper DML Frontiers (Publisher) Frontiers in Neurology 14 |
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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 |
Article in Journal/Newspaper |
author |
Li, Qian Chen, Qing Zhang, Ting Xu, Ying Kan, Yanmin Zhang, Jing |
spellingShingle |
Li, Qian Chen, Qing Zhang, Ting Xu, Ying Kan, Yanmin Zhang, Jing Case report: Anti-CNTN1 antibody-associated nodopathies disease with asymmetric onset |
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 SA |
publishDate |
2023 |
url |
http://dx.doi.org/10.3389/fneur.2023.1124540 https://www.frontiersin.org/articles/10.3389/fneur.2023.1124540/full |
genre |
DML |
genre_facet |
DML |
op_source |
Frontiers in Neurology volume 14 ISSN 1664-2295 |
op_rights |
https://creativecommons.org/licenses/by/4.0/ |
op_doi |
https://doi.org/10.3389/fneur.2023.1124540 |
container_title |
Frontiers in Neurology |
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14 |
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1802643671438327808 |