Characterization of performance fatigability in patients with facioscapulohumeral muscular dystrophy using surface EMG

Beretta Piccoli, Matteo and Calanni, Luca and Negro, Massimo and Barbero, Marco and Berardinelli, Angela and Tupler, Rossella and Soldini, Emiliano and Cescon, Corrado and D'Antona, Giuseppe (2020) Characterization of performance fatigability in patients with facioscapulohumeral muscular dystrophy using surface EMG. In: ISEK 2020 Congress - XXIII Congress of the International Society of Electrophysiology and Kinesiology, July 12-14 2020, Nagoya, Japan.

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Abstract

BACKGROUND AND AIM: In facioscapulohumeral muscular dystrophy (FSHD), the third most common hereditary myopathy, fatigue has been recognized as a critical and early symptom flag of the pathological processes leading to muscle wasting and to the incurable decline of the subjects' quality of life. Notwithstanding the clinical importance of fatigue in FSHD, its occurrence has been poorly investigated and little is known about the pathophysiology of this disabling condition. Therefore, we aimed to determine if the levels of fatigability experienced during a performance task in a sample of FSHD patients differed in comparison to healthy controls. METHODS: 18 patients diagnosed with FSHD (10 men and 8 women) were included in the study and compared to 18 matched controls. Participants performed a 20% maximal voluntary isometric contraction of 2 min, and afterwards a 60% MVC held until exhaustion. sEMG signals were detected using bi-dimensional arrays of 64 electrodes from the dominant biceps brachii. Initial values and rates of change (slope) of mean frequency of the power spectrum (MNF), conduction velocity (CV) and fractal dimension (FD) of the EMG signal were calculated. Trait levels of fatigue experienced by FSHD patients were quantified using the Checklist Individual Strength (CIS) fatigue-subscale. RESULTS: Statistically significant differences between people with FSHD and healthy controls were observed in the MVC intensity, endurance time, initial values of MNF and FD at 20% MVC (p<0.01) and in the slopes of MNF, CV and FD at 60% MVC (p<0.05) (Table 1). A correlation between perceived and performance fatigability was not determined. CONCLUSIONS: The results suggest that people with FSHD experience (1) lower levels of performance fatigability, probably related to a fast-to-slow muscle fibers transition, which is pointed out by the longer endurance time with respect to the healthy controls; and (2) a loss of muscle strength, which may justify the lower CV slope at higher degree of force production.

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