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23-09-2023 | Muscular Dystrophy | Original Communication

Face to Face: deciphering facial involvement in inclusion body myositis

Authors: Etienne Fortanier, Emilien Delmont, Ludivine Kouton, Giovanni Corazza, Aude-Marie Grapperon, Annie Verschueren, Shahram Attarian, Emmanuelle Salort-Campana

Published in: Journal of Neurology | Issue 1/2024

Abstract

Objective

The objective of this study is to evaluate the frequency and characteristics of facial involvement in inclusion body myositis (IBM) patients and to compare it to the one previously described in facioscapulohumeral dystrophy (FSHD) patients.

Methods

Thirty-two IBM patients were included and compared to 29 controls and 39 FSHD patients. All participants were recorded in a video as they performed a series of seven facial tasks. Five raters independently assessed facial weakness using both a qualitative evaluation and a semi-quantitative facial weakness score (FWS).

Results

IBM patients had higher FWS than controls (7.89 ± 7.56 vs 1.06 ± 0.88, p < 0.001). Twenty IBM patients (63%) had a facial weakness with a FWS above the maximum value for controls. All facial tasks were significantly more impaired in IBM patients compared to controls (p < 0.001), task 2 evaluating orbiculari oculi muscle weakness being the most affected. IBM patients with facial weakness reported more swallowing troubles than IBM patients without facial weakness (p = 0.03). FSHD patients displayed higher FWS than IBM patients (12.16 ± 8.37 vs 7.89 ± 7.56, p = 0.01) with more pronounced facial asymmetry (p = 0.01). FWS inter-rater ICC was 0.775.

Conclusion

This study enabled us to estimate the frequency of facial impairment in IBM in more than half of patients, to detail its characteristics and to compare them with those of FSHD patients. The standardized, semi-quantitative FWS is an interesting diagnostic help in IBM as it appeared more sensitive than qualitative evaluation to detect mild facial weakness.

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Title: Face to Face: deciphering facial involvement in inclusion body myositis
Authors: Etienne Fortanier
Emilien Delmont
Ludivine Kouton
Giovanni Corazza
Aude-Marie Grapperon
Annie Verschueren
Shahram Attarian
Emmanuelle Salort-Campana
Publication date: 23-09-2023
Publisher: Springer Berlin Heidelberg
Keywords: Muscular Dystrophy
Muscular Dystrophy
Diseases of the neuromuscular synapses and muscles
Myopathy
Published in: Journal of Neurology / Issue 1/2024
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-023-11986-7

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Face to Face: deciphering facial involvement in inclusion body myositis

Abstract

Objective

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objective

Methods

Results

Conclusion

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