Altered mechanisms of muscular force generation in lower motor neuron disease

Recruitment and firing rate modulation (FRM) of single motor units (MUs) were evaluated in the first dorsal interosseus muscle in patients with chronic lower motor neuron disorders of primarily neuroaxonal or demyelinating pathology. Residual muscle function was estimated by maximal voluntary force, twitch tension, and compound muscle action potential. The recruitment range of MUs was expanded toward higher relative force levels in all patients. Changes in firing rates per unit force increment were larger in patients with more pronounced muscle atrophy. When this effect was accounted for by calculating FRM for increments of 10% of residual maximal force, patients with subnormal motor nerve conduction velocities showed selective impairment of rate modulation. This was not due to intermittent conduction failure. We conclude that the two force-generating mechanisms, recruitment and FRM, show unspecific compensatory changes related to the loss of MUs and also alterations that are specifically related to the neuroaxonal or demyelinating nature of the neuropathy.