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Spotlight on MuSK positive myasthenia gravis: clinical characteristics, treatment and outcomes

BACKGROUND: To investigate the clinical characteristics, treatments and outcomes of patients with myasthenia gravis with antibodies to muscle-specific tyrosine kinase (MuSK-MG). METHODS: We retrospectively reviewed the cases of 21 patients with confirmed MuSK-MG between January 2012 and January 2020...

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Autores principales: Huang, Qi, Li, Feng, Zhao, Song
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8895578/
https://www.ncbi.nlm.nih.gov/pubmed/35246057
http://dx.doi.org/10.1186/s12883-022-02593-6
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author Huang, Qi
Li, Feng
Zhao, Song
author_facet Huang, Qi
Li, Feng
Zhao, Song
author_sort Huang, Qi
collection PubMed
description BACKGROUND: To investigate the clinical characteristics, treatments and outcomes of patients with myasthenia gravis with antibodies to muscle-specific tyrosine kinase (MuSK-MG). METHODS: We retrospectively reviewed the cases of 21 patients with confirmed MuSK-MG between January 2012 and January 2020 in our centre. Detailed clinical data and long-term follow-up information were summarized. RESULTS: Females (17/21, 81%) predominated among these MuSK-MG patients, and the mean age of onset in this group was 51.86 ± 16.16 years. MuSK-MG patients were divided into three subgroups according to the symptoms of muscle weakness at onset: ocular myasthenia gravis (OMG, 47.6%), bulbar myasthenia gravis (BMG, 42.9%), and generalized myasthenia gravis (GMG, 9.5%). The mean progression time from symptom onset to other muscle group involvement in OMG patients was 4.38 ± 2.54 months. Pyridostigmine bromide was adopted in 81.0% of patients, and 90.5% of patients received corticosteroids. Compared to usage in hospitals, the median daily dose of corticosteroids decreased significantly at the last follow-up. A total of 85.7% of patients received a long-term follow-up, with an average time of 1202.17 ± 976.73 days. At the end of the follow-up period, 4.8% of patients had achieved complete stable remission, 42.9% of patients had minimal manifestations, 19.0% had improved, the condition of 4.8% of patients remained unchanged, and 9.5% of patients died. CONCLUSION: Female patients were more prevalent in this study, and MuSK-MG patients rapidly progressed to a generalized state. Although approximately 50% of MuSK-MG patients can achieve a favourable outcome with conventional immunosuppressants, complete stable remission is rare, and approximately 15% respond poorly. More effective medications should be explored in these patients. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12883-022-02593-6.
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spelling pubmed-88955782022-03-10 Spotlight on MuSK positive myasthenia gravis: clinical characteristics, treatment and outcomes Huang, Qi Li, Feng Zhao, Song BMC Neurol Research BACKGROUND: To investigate the clinical characteristics, treatments and outcomes of patients with myasthenia gravis with antibodies to muscle-specific tyrosine kinase (MuSK-MG). METHODS: We retrospectively reviewed the cases of 21 patients with confirmed MuSK-MG between January 2012 and January 2020 in our centre. Detailed clinical data and long-term follow-up information were summarized. RESULTS: Females (17/21, 81%) predominated among these MuSK-MG patients, and the mean age of onset in this group was 51.86 ± 16.16 years. MuSK-MG patients were divided into three subgroups according to the symptoms of muscle weakness at onset: ocular myasthenia gravis (OMG, 47.6%), bulbar myasthenia gravis (BMG, 42.9%), and generalized myasthenia gravis (GMG, 9.5%). The mean progression time from symptom onset to other muscle group involvement in OMG patients was 4.38 ± 2.54 months. Pyridostigmine bromide was adopted in 81.0% of patients, and 90.5% of patients received corticosteroids. Compared to usage in hospitals, the median daily dose of corticosteroids decreased significantly at the last follow-up. A total of 85.7% of patients received a long-term follow-up, with an average time of 1202.17 ± 976.73 days. At the end of the follow-up period, 4.8% of patients had achieved complete stable remission, 42.9% of patients had minimal manifestations, 19.0% had improved, the condition of 4.8% of patients remained unchanged, and 9.5% of patients died. CONCLUSION: Female patients were more prevalent in this study, and MuSK-MG patients rapidly progressed to a generalized state. Although approximately 50% of MuSK-MG patients can achieve a favourable outcome with conventional immunosuppressants, complete stable remission is rare, and approximately 15% respond poorly. More effective medications should be explored in these patients. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12883-022-02593-6. BioMed Central 2022-03-04 /pmc/articles/PMC8895578/ /pubmed/35246057 http://dx.doi.org/10.1186/s12883-022-02593-6 Text en © The Author(s) 2022 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 Research
Huang, Qi
Li, Feng
Zhao, Song
Spotlight on MuSK positive myasthenia gravis: clinical characteristics, treatment and outcomes
title Spotlight on MuSK positive myasthenia gravis: clinical characteristics, treatment and outcomes
title_full Spotlight on MuSK positive myasthenia gravis: clinical characteristics, treatment and outcomes
title_fullStr Spotlight on MuSK positive myasthenia gravis: clinical characteristics, treatment and outcomes
title_full_unstemmed Spotlight on MuSK positive myasthenia gravis: clinical characteristics, treatment and outcomes
title_short Spotlight on MuSK positive myasthenia gravis: clinical characteristics, treatment and outcomes
title_sort spotlight on musk positive myasthenia gravis: clinical characteristics, treatment and outcomes
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8895578/
https://www.ncbi.nlm.nih.gov/pubmed/35246057
http://dx.doi.org/10.1186/s12883-022-02593-6
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