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Thymoma may explain the confusion: a case report

BACKGROUND: The association of inflammatory myopathy and myasthenia gravis is a rarely described entity whose clinical presentation has always been intriguing because of the great clinical similarity between these two pathologies. The presence of a thymic pathology often explains this combination, w...

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Autores principales: Maaroufi, Abdelkhaleq, Assoufi, Naoufal, Essaoudi, Mohamed Amine, Fatihi, Jamal
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8675449/
https://www.ncbi.nlm.nih.gov/pubmed/34911585
http://dx.doi.org/10.1186/s13256-021-03178-6
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author Maaroufi, Abdelkhaleq
Assoufi, Naoufal
Essaoudi, Mohamed Amine
Fatihi, Jamal
author_facet Maaroufi, Abdelkhaleq
Assoufi, Naoufal
Essaoudi, Mohamed Amine
Fatihi, Jamal
author_sort Maaroufi, Abdelkhaleq
collection PubMed
description BACKGROUND: The association of inflammatory myopathy and myasthenia gravis is a rarely described entity whose clinical presentation has always been intriguing because of the great clinical similarity between these two pathologies. The presence of a thymic pathology often explains this combination, whose mechanisms are very complex. CASE PRESENTATION: A 56-year-old woman of North African origin, was hospitalized to explore the Raynaud phenomenon associated with proximal muscle weakness, pain, and arthralgia. There was no rash, and neuromuscular examination had revealed proximal tetraparesis and mild neck weakness. Tendon reflexes were normal. There was no abnormal nail fold capillaroscopy. A significant titer of muscle enzymes had been shown on blood tests, and autoimmune screening for myositis-specific and myositis-associated autoantibodies was negative. Electromyography had shown a myopathic pattern, and muscle biopsy confirmed an inflammatory myopathy. Although steroids were introduced, the clinical course was unsatisfactory; ophthalmic and bulbar symptomatology appeared. The association of myasthenia gravis was confirmed by an elevated level of serum acetylcholine receptor. A chest computed tomography scan had identified a thymoma. Treated with prednisone, pyridostigmine, and thymectomy, the patient’s clinical and biological evolution was favorable. CONCLUSION: This case illustrates an exceptional association of two entities and the difficulty encountered during their diagnosis and treatment. The management of these two diseases is different, so it is essential to recognize this concomitant presentation.
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spelling pubmed-86754492021-12-20 Thymoma may explain the confusion: a case report Maaroufi, Abdelkhaleq Assoufi, Naoufal Essaoudi, Mohamed Amine Fatihi, Jamal J Med Case Rep Case Report BACKGROUND: The association of inflammatory myopathy and myasthenia gravis is a rarely described entity whose clinical presentation has always been intriguing because of the great clinical similarity between these two pathologies. The presence of a thymic pathology often explains this combination, whose mechanisms are very complex. CASE PRESENTATION: A 56-year-old woman of North African origin, was hospitalized to explore the Raynaud phenomenon associated with proximal muscle weakness, pain, and arthralgia. There was no rash, and neuromuscular examination had revealed proximal tetraparesis and mild neck weakness. Tendon reflexes were normal. There was no abnormal nail fold capillaroscopy. A significant titer of muscle enzymes had been shown on blood tests, and autoimmune screening for myositis-specific and myositis-associated autoantibodies was negative. Electromyography had shown a myopathic pattern, and muscle biopsy confirmed an inflammatory myopathy. Although steroids were introduced, the clinical course was unsatisfactory; ophthalmic and bulbar symptomatology appeared. The association of myasthenia gravis was confirmed by an elevated level of serum acetylcholine receptor. A chest computed tomography scan had identified a thymoma. Treated with prednisone, pyridostigmine, and thymectomy, the patient’s clinical and biological evolution was favorable. CONCLUSION: This case illustrates an exceptional association of two entities and the difficulty encountered during their diagnosis and treatment. The management of these two diseases is different, so it is essential to recognize this concomitant presentation. BioMed Central 2021-12-16 /pmc/articles/PMC8675449/ /pubmed/34911585 http://dx.doi.org/10.1186/s13256-021-03178-6 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
Maaroufi, Abdelkhaleq
Assoufi, Naoufal
Essaoudi, Mohamed Amine
Fatihi, Jamal
Thymoma may explain the confusion: a case report
title Thymoma may explain the confusion: a case report
title_full Thymoma may explain the confusion: a case report
title_fullStr Thymoma may explain the confusion: a case report
title_full_unstemmed Thymoma may explain the confusion: a case report
title_short Thymoma may explain the confusion: a case report
title_sort thymoma may explain the confusion: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8675449/
https://www.ncbi.nlm.nih.gov/pubmed/34911585
http://dx.doi.org/10.1186/s13256-021-03178-6
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