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Morvan syndrome associated with LGI1 antibody: a case report
BACKGROUND: Morvan syndrome (MoS) is a rare autoimmune syndrome associated with antibodies against two kinds of potassium channel proteins, contactin associated protein-like 2 (CASPR2) and leucine-rich glioma inactivated protein 1 (LGI1). MoS patients with only LGI1-antibody seropositivity have rare...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8091679/ https://www.ncbi.nlm.nih.gov/pubmed/33941099 http://dx.doi.org/10.1186/s12883-021-02205-9 |
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author | Zhang, Shui-Jing Xue, Yan-Yan Yu, Hao Tao, Qing-Qing |
author_facet | Zhang, Shui-Jing Xue, Yan-Yan Yu, Hao Tao, Qing-Qing |
author_sort | Zhang, Shui-Jing |
collection | PubMed |
description | BACKGROUND: Morvan syndrome (MoS) is a rare autoimmune syndrome associated with antibodies against two kinds of potassium channel proteins, contactin associated protein-like 2 (CASPR2) and leucine-rich glioma inactivated protein 1 (LGI1). MoS patients with only LGI1-antibody seropositivity have rarely been reported. Here, we describe a 64-year-old male MoS patient with only LGI1-antibody seropositivity. CASE PRESENTATION: A 64-year-old male patient was referred to our hospital due to limb pain, widespread myokymia, insomnia, constipation, and hyperhidrosis for 1 month. The patient was diagnosed with MoS based on the clinical symptoms and positive LGI1-antibody in serum. He was treated with intravenous immunoglobulin (IVIG), intravenous methylprednisolone followed by oral prednisone, and other drugs for symptomatic relief. Several days later, myokymia and insomnia symptoms improved. After 60 days of follow-up, all the drugs had been stopped for 2 weeks, and the patient achieved complete remission without any medical side effects. CONCLUSION: We report the clinical characteristics of a Chinese MoS patient with only LGI1-antibody seropositivity, and further support the view that non-neoplasm MoS patients respond well to immunotherapy. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12883-021-02205-9. |
format | Online Article Text |
id | pubmed-8091679 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-80916792021-05-04 Morvan syndrome associated with LGI1 antibody: a case report Zhang, Shui-Jing Xue, Yan-Yan Yu, Hao Tao, Qing-Qing BMC Neurol Case Report BACKGROUND: Morvan syndrome (MoS) is a rare autoimmune syndrome associated with antibodies against two kinds of potassium channel proteins, contactin associated protein-like 2 (CASPR2) and leucine-rich glioma inactivated protein 1 (LGI1). MoS patients with only LGI1-antibody seropositivity have rarely been reported. Here, we describe a 64-year-old male MoS patient with only LGI1-antibody seropositivity. CASE PRESENTATION: A 64-year-old male patient was referred to our hospital due to limb pain, widespread myokymia, insomnia, constipation, and hyperhidrosis for 1 month. The patient was diagnosed with MoS based on the clinical symptoms and positive LGI1-antibody in serum. He was treated with intravenous immunoglobulin (IVIG), intravenous methylprednisolone followed by oral prednisone, and other drugs for symptomatic relief. Several days later, myokymia and insomnia symptoms improved. After 60 days of follow-up, all the drugs had been stopped for 2 weeks, and the patient achieved complete remission without any medical side effects. CONCLUSION: We report the clinical characteristics of a Chinese MoS patient with only LGI1-antibody seropositivity, and further support the view that non-neoplasm MoS patients respond well to immunotherapy. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12883-021-02205-9. BioMed Central 2021-05-03 /pmc/articles/PMC8091679/ /pubmed/33941099 http://dx.doi.org/10.1186/s12883-021-02205-9 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Case Report Zhang, Shui-Jing Xue, Yan-Yan Yu, Hao Tao, Qing-Qing Morvan syndrome associated with LGI1 antibody: a case report |
title | Morvan syndrome associated with LGI1 antibody: a case report |
title_full | Morvan syndrome associated with LGI1 antibody: a case report |
title_fullStr | Morvan syndrome associated with LGI1 antibody: a case report |
title_full_unstemmed | Morvan syndrome associated with LGI1 antibody: a case report |
title_short | Morvan syndrome associated with LGI1 antibody: a case report |
title_sort | morvan syndrome associated with lgi1 antibody: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8091679/ https://www.ncbi.nlm.nih.gov/pubmed/33941099 http://dx.doi.org/10.1186/s12883-021-02205-9 |
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