We have clinically and electromyographically examined, during a follow-up study, weakened muscles and still clinically normal muscles of 9 patients affected by amyotrophic lateral sclerosis (ALS) and of 3 patients affected by focal benign amyotrophy (FBA). MRC scores corrected with positive semiquantitative evaluations of the number of giant motor unit (MU) action potentials and with negative evaluations of the number of fasciculations and fibrillations potentials have been found to linearly correlate with the percentage of the residual MUs calculated from the mean amplitude and the spike number during maximal voluntary contraction. A main finding is that the loss of MUs slows down in the last 10 months of the disease. it follows that after a phase when the pathological process was accelerated by the toxic effect due to the abnormally high concentration of glutamate released by dead motoneurons, the residual cells because of the decreased motoneuronal density are less exposed to the possible neurotoxic damage.
|Translated title of the contribution||Motoneuron density effect on motoneuron impoverishment curve in amyotrophic lateral sclerosis|
|Number of pages||6|
|Journal||Annali dell'Istituto Superiore di Sanita|
|Publication status||Published - 1996|
ASJC Scopus subject areas
- Public Health, Environmental and Occupational Health