Global weakness with no clear precipitant: thymoma presenting as a necrotising myositis and myasthenia gravis overlap syndrome

Overlap syndromes involving myasthenia gravis, myositis and myocarditis are recognised adverse effects of immune checkpoint inhibitors (ICI). We report a similar syndrome of myasthenia gravis and myositis in a patient without prior ICI exposure. Diagnostic tests showed signs of both immune-mediated necrotising myopathy and myasthenia gravis. Symptoms included proximal muscle weakness, ophthalmoplegia, bulbar symptoms and neuromuscular respiratory failure. Initial treatment with glucocorticoids and intravenous immunoglobulin for myositis was partially effective. Significant improvement occurred with rituximab and an acetylcholinesterase inhibitor. Although imaging had not revealed a thymic mass suggestive of thymoma, the patient underwent thymectomy several weeks after recovering from the acute illness. Histopathological examination of the resected tissue confirmed the presence of thymoma. This case illustrates that myasthenia gravis and myositis can spontaneously co-present. Clinicians should consider the possibility that a seemingly atypical presentation of a condition may reflect a more typical presentation of two conditions, such as in this case. In this situation, paraneoplastic causes should be considered.

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Publication Details

Journal
BMJ Case Reports
Published
2025-02-01
DOI
https://doi.org/10.1136/bcr-2024-262565
Primary Topic
Myasthenia Gravis and Thymoma
Type
article
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article

Global weakness with no clear precipitant: thymoma presenting as a necrotising myositis and myasthenia gravis overlap syndrome

Tamir Malley, Fernanda Ruiz, Rhys Thomas, Edmund Hugh Larkin
BMJ Case Reports
Myasthenia Gravis and Thymoma
article

Global weakness with no clear precipitant: thymoma presenting as a necrotising myositis and myasthenia gravis overlap syndrome

Tamir Malley, Fernanda Ruiz, Rhys Thomas, Edmund Hugh Larkin
article en

Abstract

Overlap syndromes involving myasthenia gravis, myositis and myocarditis are recognised adverse effects of immune checkpoint inhibitors (ICI). We report a similar syndrome of myasthenia gravis and myositis in a patient without prior ICI exposure. Diagnostic tests showed signs of both immune-mediated necrotising myopathy and myasthenia gravis. Symptoms included proximal muscle weakness, ophthalmoplegia, bulbar symptoms and neuromuscular respiratory failure. Initial treatment with glucocorticoids and intravenous immunoglobulin for myositis was partially effective. Significant improvement occurred with rituximab and an acetylcholinesterase inhibitor. Although imaging had not revealed a thymic mass suggestive of thymoma, the patient underwent thymectomy several weeks after recovering from the acute illness. Histopathological examination of the resected tissue confirmed the presence of thymoma. This case illustrates that myasthenia gravis and myositis can spontaneously co-present. Clinicians should consider the possibility that a seemingly atypical presentation of a condition may reflect a more typical presentation of two conditions, such as in this case. In this situation, paraneoplastic causes should be considered.

BMJ Case ReportsVol. 18(2)
The Royal Free Hospital (GB), Whittington Hospital (GB), University College London (GB)
Good health and well-being
Openalex Percentile: Top 96%
Myasthenia Gravis and Thymoma
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