1. Muscle Fiber Conduction Velocity Correlates With the Age at Onset in Mild FSHD Cases
- Author
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Matteo Beretta-Piccoli, Massimo Negro, Luca Calanni, Angela Berardinelli, Gabriele Siciliano, Rossella Tupler, Emiliano Soldini, Corrado Cescon, and Giuseppe D’Antona
- Subjects
musculoskeletal diseases ,0301 basic medicine ,electromyography ,medicine.medical_specialty ,Neuromuscular disease ,Physiology ,Average rectified value ,Electromyography ,Isometric exercise ,Biceps ,Nerve conduction velocity ,muscle fiber conduction velocity ,03 medical and health sciences ,0302 clinical medicine ,Physiology (medical) ,Internal medicine ,medicine ,QP1-981 ,Facioscapulohumeral muscular dystrophy ,D4Z4 ,medicine.diagnostic_test ,business.industry ,Dystrophy ,neuromuscular disease ,Brief Research Report ,medicine.disease ,correlation ,dystrophy ,fatigability ,030104 developmental biology ,Cardiology ,business ,030217 neurology & neurosurgery - Abstract
A majority of patients with facioscapulohumeral muscular dystrophy (FSHD) report severe fatigue. The aim of this study was to explore whether fatigability during a performance task is related to the main clinical features of the disease in mildly affected patients. A total of 19 individuals with a molecular genetic-based diagnosis of FSHD (median D4Z4 deletion length of 27 kb) performed two isometric flexions of the dominant biceps brachii at 20% of their maximal voluntary contraction (MVC) for 2 min, and then at 60% MVC until exhaustion. Fatigability indices (average rectified value, mean frequency, conduction velocity, and fractal dimension) were extracted from the surface electromyogram (sEMG) signal, and their correlations with age, age at onset, disease duration, D4Z4 contraction length, perceived fatigability, and clinical disability score were analyzed. The conduction velocity during the low level contraction showed a significant negative correlation with the age at onset (p < 0.05). This finding suggest the assessment of conduction velocity at low isometric contraction intensities, as a potential useful tool to highlight differences in muscle involvement in FSHD patients.
- Published
- 2021