Aim: We describe the case of a 43-year-old woman affected by Facioscapulohumeral dystrophy (FSHD) from the age of 13 with wheelchair-bound at 37 years, wasting of arm muscles, and moderate restrictive respiratory failure. Method: The resting oxygen consumption per unit time (VO2) and the VO2max were calculated by indirect calorimetry and submaximal handbyke incremental test. Maximal voluntary contraction (MVC) of muscular districts (elbow flexors, elbow extensors, shoulder abductors) was calculated. Body composition was analyzed by bioelectrical impedance analysis (BIA). Moderate endurance training (ET) was set up to 90% VO2peak (with assisted handcycling training equipment from 10min up to 20min 2 times/wk). Resistance training (RST) consisted in 2 series of 8 repetition for each muscular district 2 times/wk with elastic band (lateral abduction, biceps flexion, triceps extension with green Thera-band®). Respiratory training consisted in isocapnic hyperpnea (2min, 4 times/wk, 1.8L, 20min-1). Nutritional supplementation included a balanced essential amino acids mixture (daily 0.1 gr/kg FFM) and creatine monohydrate (0.1 gr/kg FFM) plus conjugated linoleic acid (CLA, 2.4gr) the day of RST. Results: At the end of the training period a significant clinical improvement was observed in terms of motor performance tests, body composition (reduced body mass, increased fat free mass and reduced fat mass) and a less pronounced deterioration of respiratory volumes. Conclusion: A specific integrated exercise program combined with nutritional supplementation can be safely used with beneficial effects on muscle function in patients with FSHD dystrophy.

A case report of endurance/strength training combined with nutritional supplementation in Facioscapulohumeral dystrophy

MAGNANI, BRUNO;GIOVANETTI, GIUSEPPE;D'ANTONA, GIUSEPPE
2013

Abstract

Aim: We describe the case of a 43-year-old woman affected by Facioscapulohumeral dystrophy (FSHD) from the age of 13 with wheelchair-bound at 37 years, wasting of arm muscles, and moderate restrictive respiratory failure. Method: The resting oxygen consumption per unit time (VO2) and the VO2max were calculated by indirect calorimetry and submaximal handbyke incremental test. Maximal voluntary contraction (MVC) of muscular districts (elbow flexors, elbow extensors, shoulder abductors) was calculated. Body composition was analyzed by bioelectrical impedance analysis (BIA). Moderate endurance training (ET) was set up to 90% VO2peak (with assisted handcycling training equipment from 10min up to 20min 2 times/wk). Resistance training (RST) consisted in 2 series of 8 repetition for each muscular district 2 times/wk with elastic band (lateral abduction, biceps flexion, triceps extension with green Thera-band®). Respiratory training consisted in isocapnic hyperpnea (2min, 4 times/wk, 1.8L, 20min-1). Nutritional supplementation included a balanced essential amino acids mixture (daily 0.1 gr/kg FFM) and creatine monohydrate (0.1 gr/kg FFM) plus conjugated linoleic acid (CLA, 2.4gr) the day of RST. Results: At the end of the training period a significant clinical improvement was observed in terms of motor performance tests, body composition (reduced body mass, increased fat free mass and reduced fat mass) and a less pronounced deterioration of respiratory volumes. Conclusion: A specific integrated exercise program combined with nutritional supplementation can be safely used with beneficial effects on muscle function in patients with FSHD dystrophy.
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11571/760837
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