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Face to Face: deciphering facial involvement in inclusion body myositis. 2024

Etienne Fortanier, and Emilien Delmont, and Ludivine Kouton, and Giovanni Corazza, and Aude-Marie Grapperon, and Annie Verschueren, and Shahram Attarian, and Emmanuelle Salort-Campana
Referral Centre for Neuromuscular Diseases and ALS (Amyotrophic Lateral Sclerosis), Timone University Hospital, Marseille, France. etienne.fortanier@ap-hm.fr.

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. CONCLUSIONS 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.

UI MeSH Term Description Entries
D009220 Myositis Inflammation of a muscle or muscle tissue. Inflammatory Myopathy,Myositis, Focal,Myositis, Infectious,Idiopathic Inflammatory Myopathies,Idiopathic Inflammatory Myopathy,Idiopathic Inflammatory Myositis,Infectious Myositis,Inflammatory Muscle Diseases,Inflammatory Myopathies, Idiopathic,Inflammatory Myopathy, Idiopathic,Muscle Diseases, Inflammatory,Myopathies, Idiopathic Inflammatory,Myopathy, Inflammatory,Myositis, Proliferative,Focal Myositides,Focal Myositis,Infectious Myositides,Inflammatory Muscle Disease,Inflammatory Myopathies,Muscle Disease, Inflammatory,Myopathies, Inflammatory,Myopathy, Idiopathic Inflammatory,Myositides,Myositides, Focal,Myositides, Infectious,Myositides, Proliferative,Proliferative Myositides,Proliferative Myositis
D003679 Deglutition The act of taking solids and liquids into the GASTROINTESTINAL TRACT through the mouth and throat. Swallowing,Deglutitions,Swallowings
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D018979 Myositis, Inclusion Body Progressive myopathies characterized by the presence of inclusion bodies on muscle biopsy. Sporadic and hereditary forms have been described. The sporadic form is an acquired, adult-onset inflammatory vacuolar myopathy affecting proximal and distal muscles. Familial forms usually begin in childhood and lack inflammatory changes. Both forms feature intracytoplasmic and intranuclear inclusions in muscle tissue. (Adams et al., Principles of Neurology, 6th ed, pp1409-10) Inclusion Body Myositis,Myopathy, Inclusion Body, Sporadic,Inclusion Body Myopathy, Sporadic,Inclusion Body Myositis, Sporadic,Myositis, Inclusion Body, Sporadic,Sporadic Inclusion Body Myositis,Inclusion Body Myositides,Myositides, Inclusion Body
D020391 Muscular Dystrophy, Facioscapulohumeral An autosomal dominant degenerative muscle disease characterized by slowly progressive weakness of the muscles of the face, upper-arm, and shoulder girdle. The onset of symptoms usually occurs in the first or second decade of life. Affected individuals usually present with impairment of upper extremity elevation. This tends to be followed by facial weakness, primarily involving the orbicularis oris and orbicularis oculi muscles. (Neuromuscul Disord 1997;7(1):55-62; Adams et al., Principles of Neurology, 6th ed, p1420) Facioscapulohumeral Muscular Dystrophy,Landouzy-Dejerine Dystrophy,FSH Muscular Dystrophy,Facio-Scapulo-Humeral Dystrophy,Facioscapulohumeral Atrophy,Facioscapulohumeral Type Progressive Muscular Dystrophy,Facioscapuloperoneal Muscular Dystrophy,Muscular Dystrophy, Landouzy Dejerine,Progressive Muscular Dystrophy, Facioscapulohumeral Type,Atrophies, Facioscapulohumeral,Atrophy, Facioscapulohumeral,Dystrophies, Facioscapulohumeral Muscular,Dystrophies, Landouzy-Dejerine,Dystrophy, Facioscapulohumeral Muscular,Dystrophy, Landouzy-Dejerine,Facioscapulohumeral Atrophies,Facioscapulohumeral Muscular Dystrophies,Landouzy Dejerine Dystrophy,Landouzy-Dejerine Dystrophies,Muscular Dystrophies, Facioscapulohumeral

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