In 22 patients suffering from progressive muscular dystrophy (PMD), EMG recordings (measurement of amplitude of volitional action potentials, of the M-wave evoked by supramaximal stimulation of appropriate peripheral nerves and of the H-wave) were obtained from the tibialis anterior, gastrocnemius and soleus muscles in addition to manual examinations of muscular strength. Twelve patients had been diagnosed as suffering from the Duchenne type dystrophy and 10 were classified in the limb-girdle category on the basis of clinical and genetic criteria. Clinical and electromyographic analysis showed that the dystrophic process affected differentially the tibialis anterior, gastrocnemius and soleus. It was possible to demonstrate early electromyographic changes in the tibialis anterior both in the benign limb-girdle type and in the severe Duchenne type of PMD. The electromyographic changes consisted of the characteristic “dystrophic” type of the M-wave and of a diminution in amplitude of the interference pattern and of the M-wave. On the other hand the soleus was well preserved even at a late stage of the disease both in the limb-girdle and Duchenne types. The soleus is eventually involved too in certain very advanced cases of the Duchenne type. When affected, the soleus also shows the characteristic “dystrophic” type of the M-wave, diminution in amplitude of the interference pattern, of the M-wave and of the action potential of tendon jerk, but it continues to produce an H-wave. These findings confirm that the dystrophic process does not affect equally all types of extrafusal muscle fibres. The predominantly tonic soleus is less vulnerable to the dystrophic process than is the mixed tonic and phasic gastrocnemius, while both are less vulnerable than the predominantly phasic tibialis anterior. PMD cannot therefore be defined simply as a degenerative disease of the muscles, but appears to be a selective degenerative disorder affecting only extrafusal muscle fibres; and among these those primarily involved appear to belong to phasic motor units.
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