Electromechanical coupling and synchronous firing of single wrist extensor motor units in sporadic amyotrophic lateral sclerosis.
Electrical and contractile properties of motor units (MU) were studied in the extensor carpi radialis muscles during voluntary contraction. The discharge of 234 single MUs was recorded in 11 patients with sporadic amyotrophic lateral sclerosis (ALS) and compared with that of the 260 MUs recorded in 12 healthy control subjects. Characteristics of the MU twitches and of the macro-potentials, the electromechanical coupling and the synchronization of the motor neurone discharges, were compared. In 5 patients (population ALS1), the twitch contraction force and macro-MUP area values were much larger than those of the controls. In the 6 other patients (population ALS2), the twitch force was considerably depressed, whereas the macro-MUP area was slightly, but significantly, increased. In ALS1, as well as in ALS2, the electromechanical coupling was much weaker than in the controls, and the fast-contracting MUs were more severely affected than the slowly contracting MUs. The motoneuronal synchronization was assessed by performing cross-correlation analysis on MUs discharges, and was used as an index to the strength of the common motoneuronal inputs. The rate of occurrence of synchronous firing was conspicuously lower in both populations of patients than in the control group. This might reflect the loss of corticospinal projections that occurs in ALS. The data are discussed in terms of the time course of motor neurone axonal sprouting, and in terms of the neuronal and muscular dysfunction possibly involved in ALS disease.
Schmied
A
A
Physiologie et Physiopathologie Neuromusculaire Humaine NBM-CNRS, Marseille, France.
Pouget
J
J
Vedel
J P
JP
D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000503:physiopathology*; D000704:Analysis of Variance; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology*; D009132:Muscles / Q000502:physiology*