Motor unit number estimation (mune) as a quantitative measure of disease progression and motor unit reorganization in amyotrophic lateral sclerosis.
Motor Unit Number Estimation (MUNE), a technique allowing to estimate the number of functioning Motor Units (MU) in single muscles, was used to score the disease's severity and progression rate in a group of 58 patients with Amyotrophic Lateral Sclerosis (ALS). All patients underwent MUNE in the abductor digiti minimi (ADM) muscle during the diagnostic work-up (T0), after three (T1) and six (T2) months. A significant loss [p < .001] of MU and a decrease [p < .001] of the maximal M wave area at T0 was found, whereas mean step area was increased [p < .001]. During the follow-up (T1 and T2), MU loss continued, maximal M wave decreased, and mean step area increased significantly. The results confirm that MUNE is a suitable tool to quantify the pathological changes in MU in patients with ALS.
Sartucci
F
F
Department of Neuroscience, Clinical Neurophysiology, Pisa University Medical School, Pisa, Italy. f.sartucci@neuro.med.unipi.it
Maritato
P
P
Moscato
G
G
Orlandi
G
G
Calabrese
R
R
Domenici
G L
GL
Murri
L
L
D000200:Action Potentials / Q000502:physiology; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology* / Q000503:physiopathology; D018450:Disease Progression; D004558:Electric Stimulation; D004576:Electromyography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000473:pathology* / Q000502:physiology; D009132:Muscles / Q000166:cytology / Q000294:innervation*