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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ftdoajarticles:oai:doaj.org/article:e2853b4d6961425baebc0be7f5ff5861 2023-05-15T16:01:57+02:00 Case report: Anti-CNTN1 antibody-associated nodopathies disease with asymmetric onset Qian Li Qing Chen Ting Zhang Ying Xu Yanmin Kan Jing Zhang 2023-03-01T00:00:00Z https://doi.org/10.3389/fneur.2023.1124540 https://doaj.org/article/e2853b4d6961425baebc0be7f5ff5861 EN eng Frontiers Media S.A. https://www.frontiersin.org/articles/10.3389/fneur.2023.1124540/full https://doaj.org/toc/1664-2295 1664-2295 doi:10.3389/fneur.2023.1124540 https://doaj.org/article/e2853b4d6961425baebc0be7f5ff5861 Frontiers in Neurology, Vol 14 (2023) contactin-1 Ranvier's autoimmune nodopathies paranodal peripheral neuropathy Neurology. Diseases of the nervous system RC346-429 article 2023 ftdoajarticles https://doi.org/10.3389/fneur.2023.1124540 2023-03-12T01:32:29Z 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 Directory of Open Access Journals: DOAJ Articles Frontiers in Neurology 14 |
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Directory of Open Access Journals: DOAJ Articles |
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ftdoajarticles |
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English |
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contactin-1 Ranvier's autoimmune nodopathies paranodal peripheral neuropathy Neurology. Diseases of the nervous system RC346-429 |
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contactin-1 Ranvier's autoimmune nodopathies paranodal peripheral neuropathy Neurology. Diseases of the nervous system RC346-429 Qian Li Qing Chen Ting Zhang Ying Xu Yanmin Kan Jing Zhang Case report: Anti-CNTN1 antibody-associated nodopathies disease with asymmetric onset |
topic_facet |
contactin-1 Ranvier's autoimmune nodopathies paranodal peripheral neuropathy Neurology. Diseases of the nervous system RC346-429 |
description |
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 |
Qian Li Qing Chen Ting Zhang Ying Xu Yanmin Kan Jing Zhang |
author_facet |
Qian Li Qing Chen Ting Zhang Ying Xu Yanmin Kan Jing Zhang |
author_sort |
Qian Li |
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 |
https://doi.org/10.3389/fneur.2023.1124540 https://doaj.org/article/e2853b4d6961425baebc0be7f5ff5861 |
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DML |
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DML |
op_source |
Frontiers in Neurology, Vol 14 (2023) |
op_relation |
https://www.frontiersin.org/articles/10.3389/fneur.2023.1124540/full https://doaj.org/toc/1664-2295 1664-2295 doi:10.3389/fneur.2023.1124540 https://doaj.org/article/e2853b4d6961425baebc0be7f5ff5861 |
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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1766397617867063296 |