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Face to Face: deciphering facial involvement in inclusion body myositis

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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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Authors and Affiliations

  1. Referral Centre for Neuromuscular Diseases and ALS (Amyotrophic Lateral Sclerosis), Timone University Hospital, Marseille, France

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

  2. Aix-Marseille University, Inserm, GMGF, Marseille, France

    Shahram Attarian & Emmanuelle Salort-Campana

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  1. Etienne Fortanier
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Correspondence to Etienne Fortanier.

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This study was approved by the Ethics Committee of La Timone (reference RGPD 2019–01 PADS22-38) and conducted in compliance with the Declaration of Helsinki.

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Fortanier, E., Delmont, E., Kouton, L. et al. Face to Face: deciphering facial involvement in inclusion body myositis. J Neurol 271, 410–418 (2024). https://doi.org/10.1007/s00415-023-11986-7

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