Mechanical and electrical responses of intact thenar muscles to indirect stimuli

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Abstract
Objectively measured differences in skeletal muscle exist between patients with pseudohypertrophic muscular dystrophy and healthy subjects of the same age and sex. Skeletal muscle weakness is associated with slowing of contraction and relaxation, decreased stiffness, and inability to sustain tension during and after induced repetitive activity. From the data, we conclude that, in pseudohypertrophic muscular dystrophy: (1) Weakness of intrinsic hand muscles can be objectively measured early in the disease. (2) The weakness of the muscle results from a decreased number of contracting muscle fibers and inherent decreased ability of the contractile tissue to generate tension. (3) The elastic properties ^of the muscle (contractile and/or noncontractile tissue) are changed so that the muscle is less stiff. This change in extensibility differs from that in mammalian denervated and immobilized muscle. (4) Different characteristics, particularly with regard to stiffness and the events after tetanus, of the muscle in pseudohypertrophic muscular dystrophy and those reported in genetic dystrophic mouse muscle suggest that the disease process differs in these two conditions and emphasizes the fact that human dystrophic muscle must be studied in order to elucidate the disease process.

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