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MRI evidence of extraocular muscle atrophy and fatty replacement in myasthenia gravis

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Abstract

Purpose

The aim of the study was to evaluate extraocular muscle (EOM) atrophy and fatty replacement in ocular myasthenia gravis (OMG) and generalized myasthenia gravis (GMG) patients with chronic and untreated ocular symptoms or with inadequate response to immunotherapy and unprovoked ocular exacerbations despite chronic immunotherapy.

Methods

Nineteen patients with either OMG or GMG and 19 healthy age-matched controls underwent an orbital MRI. Visually obvious muscle atrophy and muscle fatty replacement were evaluated by two raters independently. Maximum thickness of EOM was measured. Measurements of the muscles of each participant were added up, in order to calculate the total thickness.

Results

Eleven patients suffered from AChR-positive GMG, and 8 patients from OMG. All patients had chronic ocular symptoms or inadequate response to corticosteroids and unprovoked ocular exacerbations in spite of immunotherapy. Fatty replacement was reported in 6/19 (31.6%) patients and 0/19 (0%) controls (p = 0.02). Obvious atrophy in at least one muscle was reported in 8/19 (42.1%) patients and 1/19 (5.3%) controls (p = 0.019). Statistically significant differences between the two groups were also found in the mean total thickness, as well as in the thickness of superior recti, levator palpebrae, inferior recti, and superior oblique muscles.

Conclusion

EOM atrophy and fatty replacement were seen frequently in our series of MG patients with treatment difficulties and frequent relapses of ocular involvement.

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Data availability

The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

Code availability

Not applicable.

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Acknowledgements

We thank all patients and healthy controls for their willingness to participate in the present study.

Funding

This study did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author information

Authors and Affiliations

Authors

Contributions

All authors contributed to the study conception and design. Material preparation and data collection were performed by G Velonakis, V Papadopoulos, and V Zouvelou. Data evaluation and analysis were performed by all authors. The first draft of the manuscript was written by G Velonakis and V Zouvelou, and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Vasiliki Zouvelou.

Ethics declarations

Conflict of interest

G Velonakis, V Papadopoulos, E Karavasilis, D Filippiadisa, and V Zouvelou declare no competing interests.

Ethics approval

This cross-sectional study was approved by the local ethical committee (Eginition Hospital – National and Kapodistrian University of Athens) and conducted according to the Declaration of Helsinki and its later amendments.

Consent to participate

All patients and healthy controls provided written informed consent for their participation in the study.

Consent for publication

Written consent was also obtained from all patients whose MRI images are included in this manuscript.

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Velonakis, G., Papadopoulos, V.E., Karavasilis, E. et al. MRI evidence of extraocular muscle atrophy and fatty replacement in myasthenia gravis. Neuroradiology 63, 1531–1538 (2021). https://doi.org/10.1007/s00234-021-02753-4

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  • DOI: https://doi.org/10.1007/s00234-021-02753-4

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