A longitudinal study of the pathophysiological changes in single human thenar motor units in amyotrophic lateral sclerosis.
The sequence of pathophysiological changes in amyotrophic lateral sclerosis (ALS) at the single motor unit (MU) level is not well understood. Using a recently described technique, a comprehensive range of physiological properties in two thenar MUs in ALS were intensively studied. In the first MU, despite a marked decline in the ability of the subject to voluntarily recruit the MU, the physiological properties of this MU remained remarkably stable over a 2-year period. In contrast, the physiological properties of the other MU declined rapidly over 5 months despite the fact that this MU could be recruited with ease throughout the study period. These differences between the progressively dysfunctional changes in these two MUs illustrates the value of such longitudinal studies of specific MUs in improving our understanding of the evolution of changes in single motoneurons in ALS. The broader application of longitudinally tracking the pathophysiological changes of the surviving MUs may prove to be a sensitive measure of disease progression and in evaluating the effectiveness of treatments.
Chan
K M
KM
Department of Neurology, New England Medical Center, Boston, Massachusetts 02111, USA.
Stashuk
D W
DW
Brown
W F
WF
D000200:Action Potentials / Q000502:physiology; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000503:physiopathology*; D018450:Disease Progression; D004558:Electric Stimulation; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology*; D018482:Muscle, Skeletal / Q000294:innervation*; D011999:Recruitment, Neurophysiological / Q000502:physiology; D013933:Thumb