Clinical and diagnostic features of anti‐neurofascin‐155 antibody‐positive neuropathy in Han Chinese

Abstract Objective To investigate the clinical features of Han Chinese patients with anti‐neurofascin‐155 (NF155) antibody‐positive neuropathy. Methods We screened 194 patients with peripheral neuropathy for NF155 antibodies using a cell‐based assay (CBA) and teased‐fiber immunofluorescence assay. W...

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Main Authors: Wenqing Wang, Chang Liu, Wei Li, Dong Zhang, Yi Shan, Jinfan Zheng, Jingli Shan, Yuying Zhao, Chuanzhu Yan, Qinzhou Wang
Format: Article
Language:English
Published: Wiley 2022-05-01
Series:Annals of Clinical and Translational Neurology
Online Access:https://doi.org/10.1002/acn3.51550
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author Wenqing Wang
Chang Liu
Wei Li
Dong Zhang
Yi Shan
Jinfan Zheng
Jingli Shan
Yuying Zhao
Chuanzhu Yan
Qinzhou Wang
author_facet Wenqing Wang
Chang Liu
Wei Li
Dong Zhang
Yi Shan
Jinfan Zheng
Jingli Shan
Yuying Zhao
Chuanzhu Yan
Qinzhou Wang
author_sort Wenqing Wang
collection DOAJ
description Abstract Objective To investigate the clinical features of Han Chinese patients with anti‐neurofascin‐155 (NF155) antibody‐positive neuropathy. Methods We screened 194 patients with peripheral neuropathy for NF155 antibodies using a cell‐based assay (CBA) and teased‐fiber immunofluorescence assay. We summarized the clinical findings of seropositive patients. Results The sera from 17 patients reacted to human embryonic kidney 293 cells transfected with NF155. Eleven of these patients had the immunoglobulin G (IgG) 4 isotype, a younger onset age, tremor, higher levels of cerebrospinal fluid protein, a larger diameter of the lumbosacral nerve root on magnetic resonance imaging, and the distal demyelinating symmetric phenotype. Most patients responded to steroids and rituximab. For the remaining six seropositive patients in CBA, the predominant antibody isotype was IgG3, IgG1, or undetectable, and only one patient with IgG3 showed a positive result in the teased‐fiber immunofluorescence assay. These patients did not share the typical features displayed by patients with the IgG4 isotype. Interpretation In the Han Chinese population, a significant proportion of patients who fulfilled the criteria for chronic inflammatory demyelinating polyradiculoneuropathy diagnosis had anti‐NF155 IgG4 antibody‐positive neuropathy and displayed specific phenotypes. Ambiguous staining patterns may appear, and the potential for false positivity should be considered. For patients who presented with specific phenotypes, identifying antibodies and subtypes involved a significant laboratory workup.
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spelling doaj.art-01df2d992b134a81b561bc4ee9a026732022-12-22T00:12:16ZengWileyAnnals of Clinical and Translational Neurology2328-95032022-05-019569570610.1002/acn3.51550Clinical and diagnostic features of anti‐neurofascin‐155 antibody‐positive neuropathy in Han ChineseWenqing Wang0Chang Liu1Wei Li2Dong Zhang3Yi Shan4Jinfan Zheng5Jingli Shan6Yuying Zhao7Chuanzhu Yan8Qinzhou Wang9Research Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Cheeloo College of Medicine Shandong University Jinan ChinaResearch Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Cheeloo College of Medicine Shandong University Jinan ChinaResearch Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Cheeloo College of Medicine Shandong University Jinan ChinaResearch Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Cheeloo College of Medicine Shandong University Jinan ChinaResearch Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Cheeloo College of Medicine Shandong University Jinan ChinaResearch Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Cheeloo College of Medicine Shandong University Jinan ChinaResearch Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Cheeloo College of Medicine Shandong University Jinan ChinaResearch Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Cheeloo College of Medicine Shandong University Jinan ChinaResearch Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Cheeloo College of Medicine Shandong University Jinan ChinaResearch Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Cheeloo College of Medicine Shandong University Jinan ChinaAbstract Objective To investigate the clinical features of Han Chinese patients with anti‐neurofascin‐155 (NF155) antibody‐positive neuropathy. Methods We screened 194 patients with peripheral neuropathy for NF155 antibodies using a cell‐based assay (CBA) and teased‐fiber immunofluorescence assay. We summarized the clinical findings of seropositive patients. Results The sera from 17 patients reacted to human embryonic kidney 293 cells transfected with NF155. Eleven of these patients had the immunoglobulin G (IgG) 4 isotype, a younger onset age, tremor, higher levels of cerebrospinal fluid protein, a larger diameter of the lumbosacral nerve root on magnetic resonance imaging, and the distal demyelinating symmetric phenotype. Most patients responded to steroids and rituximab. For the remaining six seropositive patients in CBA, the predominant antibody isotype was IgG3, IgG1, or undetectable, and only one patient with IgG3 showed a positive result in the teased‐fiber immunofluorescence assay. These patients did not share the typical features displayed by patients with the IgG4 isotype. Interpretation In the Han Chinese population, a significant proportion of patients who fulfilled the criteria for chronic inflammatory demyelinating polyradiculoneuropathy diagnosis had anti‐NF155 IgG4 antibody‐positive neuropathy and displayed specific phenotypes. Ambiguous staining patterns may appear, and the potential for false positivity should be considered. For patients who presented with specific phenotypes, identifying antibodies and subtypes involved a significant laboratory workup.https://doi.org/10.1002/acn3.51550
spellingShingle Wenqing Wang
Chang Liu
Wei Li
Dong Zhang
Yi Shan
Jinfan Zheng
Jingli Shan
Yuying Zhao
Chuanzhu Yan
Qinzhou Wang
Clinical and diagnostic features of anti‐neurofascin‐155 antibody‐positive neuropathy in Han Chinese
Annals of Clinical and Translational Neurology
title Clinical and diagnostic features of anti‐neurofascin‐155 antibody‐positive neuropathy in Han Chinese
title_full Clinical and diagnostic features of anti‐neurofascin‐155 antibody‐positive neuropathy in Han Chinese
title_fullStr Clinical and diagnostic features of anti‐neurofascin‐155 antibody‐positive neuropathy in Han Chinese
title_full_unstemmed Clinical and diagnostic features of anti‐neurofascin‐155 antibody‐positive neuropathy in Han Chinese
title_short Clinical and diagnostic features of anti‐neurofascin‐155 antibody‐positive neuropathy in Han Chinese
title_sort clinical and diagnostic features of anti neurofascin 155 antibody positive neuropathy in han chinese
url https://doi.org/10.1002/acn3.51550
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