D Bachasson1, J Temesi2, C Bankole3, E Lagrange4, C Boutte4, G Y Millet5, S Verges6, P Levy1, L Feasson7, B Wuyam1. 1. 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. 2. Université de Lyon, Laboratoire de Physiologie de l'Exercice, F-42023 Saint-Etienne, France. 3. 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. 4. 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. 5. INSERM, U1042, F-38000 Grenoble, France; Université de Lyon, Laboratoire de Physiologie de l'Exercice, F-42023 Saint-Etienne, France. 6. Université Joseph Fourier, Laboratoire HP2, F-38000 Grenoble, France; INSERM, U1042, F-38000 Grenoble, France. Electronic address: sverges@chu-grenoble.fr. 7. 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.
Abstract
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.
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 CMTpatients. Supramaximal FNMS was achieved in ∼70% of FSHDpatients and in no CMTpatients. In FSHDpatients, 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.
Authors: Louise A Beveridge; Rosemary J G Price; Louise A Burton; Miles D Witham; Allan D Struthers; Deepa Sumukadas Journal: BMC Res Notes Date: 2018-06-15
Authors: Adam J Bittel; Sen Chandra Sreetama; Daniel C Bittel; Adam Horn; James S Novak; Toshifumi Yokota; Aiping Zhang; Rika Maruyama; Kenji Rowel Q Lim; Jyoti K Jaiswal; Yi-Wen Chen Journal: Int J Mol Sci Date: 2020-08-04 Impact factor: 5.923
Authors: Matthew F Jacques; Gladys L Onambele-Pearson; Neil D Reeves; Georgina K Stebbings; Jonathon Smith; Christopher I Morse Journal: J Cachexia Sarcopenia Muscle Date: 2018-10-19 Impact factor: 12.910
Authors: Saskia Lassche; Nicol C Voermans; Tim Schreuder; Arend Heerschap; Benno Küsters; Coen Ac Ottenheijm; Maria Te Hopman; Baziel Gm van Engelen Journal: Muscle Nerve Date: 2020-10-15 Impact factor: 3.217