The effects of strength training in patients with selected neuromuscular disorders.

Five subjects with spinal muscular atrophy, limb-girdle or facioscapulohumeral muscular dystrophy, were studied. Measurements pre- and post-training included: maximum isometric, dynamic and isokinetic strength, in single-arm curl and double-leg press exercises; contractile properties of the elbow flexors; computerized tomography of the upper arms and thighs; muscle biopsies from the biceps brachii muscle of each arm in three subjects. Dynamic weight training was performed 3 times per week for 9 wk; exercises comprised unilateral arm curls (the contralateral arm acted as a control), and bilateral leg press. Strength increases in the trained arm were between 19 and 34%, and from -14 to +25% in the control arm; leg strength increased from 11 to 50%. Moreover, the pretraining maximum load could be lifted from 3 to 48 times in the trained limbs, and from 1 to 13 times in an untrained limb before fatigue. Contractile properties of the elbow flexors were unchanged with training, but pre-intervention, three subjects demonstrated incomplete motor unit activation. Most of the gains in strength were apparently due to a neural adaptation, rather than muscle hypertrophy. The tomograms and biopsy samples were inadequate to determine muscle, or muscle fiber areas with confidence; they did indicate however, no additional overt muscle structural damage. Strength training may be a potentially useful therapeutic option in the management of selected neuromuscular disorders.