title: Assessement of quadriceps strength, endurance and fatigue in FSHD and CMT: benefits and limits of femoral nerve magnetic stimulation.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  This test may be a valuable tool for patient follow-up although further development of magnetic stimulation devices is needed to extend its applicability.
authors.affiliation: Université Joseph Fourier, Laboratoire HP2, F-38000 Grenoble, France; INSERM, U1042, F-38000 Grenoble, France; CHU de Grenoble, Pole Locomotion, Rééducation & Physiologie, Clinique Physiologie, Sommeil et Exercice, F-38000 Grenoble, France.
authors.forename: D
authors.initials: D
authors.lastname: Bachasson
authors.affiliation: Université de Lyon, Laboratoire de Physiologie de l'Exercice, F-42023 Saint-Etienne, France.
authors.forename: J
authors.initials: J
authors.lastname: Temesi
authors.affiliation: Université de Lyon, Laboratoire de Physiologie de l'Exercice, F-42023 Saint-Etienne, France; CHU de St Etienne, Unité de Myologie, F-42055 St. Etienne, France.
authors.forename: C
authors.initials: C
authors.lastname: Bankole
authors.affiliation: CHU de Grenoble, Pole Locomotion, Rééducation & Physiologie, Clinique Physiologie, Sommeil et Exercice, F-38000 Grenoble, France; Centres Référent Maladies Neuromusculaires Rares Rhône Alpes, CHU de Grenoble, F-38000 Grenoble, France.
authors.forename: E
authors.initials: E
authors.lastname: Lagrange
authors.affiliation: CHU de Grenoble, Pole Locomotion, Rééducation & Physiologie, Clinique Physiologie, Sommeil et Exercice, F-38000 Grenoble, France; Centres Référent Maladies Neuromusculaires Rares Rhône Alpes, CHU de Grenoble, F-38000 Grenoble, France.
authors.forename: C
authors.initials: C
authors.lastname: Boutte
authors.affiliation: INSERM, U1042, F-38000 Grenoble, France; Université de Lyon, Laboratoire de Physiologie de l'Exercice, F-42023 Saint-Etienne, France.
authors.forename: G Y
authors.initials: GY
authors.lastname: Millet
authors.affiliation: Université Joseph Fourier, Laboratoire HP2, F-38000 Grenoble, France; INSERM, U1042, F-38000 Grenoble, France. Electronic address: sverges@chu-grenoble.fr.
authors.forename: S
authors.initials: S
authors.lastname: Verges
authors.affiliation: Université Joseph Fourier, Laboratoire HP2, F-38000 Grenoble, France; INSERM, U1042, F-38000 Grenoble, France; CHU de Grenoble, Pole Locomotion, Rééducation & Physiologie, Clinique Physiologie, Sommeil et Exercice, F-38000 Grenoble, France.
authors.forename: P
authors.initials: P
authors.lastname: Levy
authors.affiliation: Université de Lyon, Laboratoire de Physiologie de l'Exercice, F-42023 Saint-Etienne, France; Centres Référent Maladies Neuromusculaires Rares Rhône Alpes, CHU de Grenoble, F-38000 Grenoble, France; CHU de St Etienne, Unité de Myologie, F-42055 St. Etienne, France.
authors.forename: L
authors.initials: L
authors.lastname: Feasson
authors.affiliation: Université Joseph Fourier, Laboratoire HP2, F-38000 Grenoble, France; INSERM, U1042, F-38000 Grenoble, France; CHU de Grenoble, Pole Locomotion, Rééducation & Physiologie, Clinique Physiologie, Sommeil et Exercice, F-38000 Grenoble, France.
authors.forename: B
authors.initials: B
authors.lastname: Wuyam
mesh_terms: D000328:Adult; D002607:Charcot-Marie-Tooth Disease / Q000503:physiopathology*; D004576:Electromyography; D005240:Feasibility Studies; D005260:Female; D005267:Femoral Nerve / Q000502:physiology*; D006801:Humans; D007537:Isometric Contraction / Q000502:physiology; D008280:Magnetics; D008297:Male; D008875:Middle Aged; D018763:Muscle Fatigue / Q000502:physiology*; D053580:Muscle Strength / Q000502:physiology*; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000503:physiopathology*; D052097:Quadriceps Muscle / Q000294:innervation / Q000503:physiopathology*; D015203:Reproducibility of Results
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