Objectives: To (i) evaluate the feasibility and the reliability of a test assessing quadriceps strength, endurance and fatigue in patients with fascioscapulohumeral dystrophy (FSHD) and Charcot-Marie-Tooth disease (CMT), (ii) compare quadriceps function between patients and healthy controls.
Methods: Controls performed the test once and patients twice on two separate visits. It involved progressive sets of 10 isometric contractions each followed by neuromuscular assessments with FNMS.
Results: Volitional assessment of muscle strength, endurance and fatigue appeared to be reliable in FSHD and CMT patients. Supramaximal FNMS was achieved in ∼70% of FSHD patients and in no CMT patients. In FSHD patients, Femoral nerve magnetic stimulation (FNMS) provided reliable assessment of central (typical error as a coefficient of variation (CVTE)<8% for voluntary activation) and peripheral (CVTE<10% and intraclass coefficient correlation >0.85 for evoked responses) function. Patients and controls had similar reductions in evoked quadriceps responses, voluntary activation and similar endurance.
Conclusions: This test provides reliable evaluation but FNMS exhibits limitations due to insufficient stimulation intensity particularly in neurogenic conditions. It showed similar central and peripheral quadriceps fatigability in patients and controls.
Significance: This test may be a valuable tool for patient follow-up although further development of magnetic stimulation devices is needed to extend its applicability.
Keywords: 10-Hz potentiated doublets; 100-Hz potentiated doublets; CMT; Charcot-Marie-Tooth disease; Db(10); Db(10):Db(100); Db(100); Experienced fatigue; FNMS; FSHD; Fascioscapulohumeral dystrophy; Femoral magnetic nerve stimulation; Hereditary motor and sensory neuropathy; MVC; MVC(RMS/M); Muscle endurance; Muscle fatigue; Muscle strength; Neuromuscular diseases; SF-36; Tw(p); VA; fascioscapulohumeral dystrophy; femoral nerve magnetic stimulation; maximal voluntary activation; maximal voluntary contraction; medical outcomes study short-form; peak potentiated 10-Hz doublets/peak potentiated 100-Hz doublets; potentiated single twitch; root mean squared calculated from vastus lateralis EMG signal normalized to M-wave amplitude during maximal voluntary contraction.
Copyright © 2013 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.