Longitudinal study of fiber density and motor unit number estimate in patients with amyotrophic lateral sclerosis.
We examined fiber density, compound muscle action potential (CMAP) amplitude, and motor unit number estimate (MUNE) of the abductor digiti minimi and grip strength longitudinally. We sought to determine the effects of ALS on these measurements and to evaluate which of these tests may be more sensitive in evaluating progression of ALS and possibly predicting survival. Ten patients were examined at months 0, 3, and 6. A significant decrease in MUNE and increase in fiber density were observed at months 3 and 6 (p < 0.02) compared with baseline (month 0). Mean CMAP and grip strength declined, but not significantly. The decrease in MUNE over 6 months was significantly greater than that of CMAP and grip strength (p < 0.025). The significant changes in MUNE and fiber density over time suggest that they are more sensitive in measuring the rate of progression of ALS. To evaluate further the utility of these tests, we arbitrarily divided the patients into equal groups based on length of survival. MUNE declined significantly in the group with shorter survival (p < 0.01). Conversely, fiber density increased significantly in patients with longer survival (p < 0.01). With similar statistical analysis there were no significant differences in decline of CMAP or grip strength in either subgroup over 6 months. Our study suggests that MUNE and fiber density are more sensitive than CMAP and grip strength in detecting progression of ALS. Furthermore, we raise the hypotheses that a greater increase in fiber density identifies a group of patients with ALS who will have longer survival, and that a greater decline in MUNE identifies a group with a worse prognosis.
Yuen
E C
EC
Department of Neurology, University of Washington, Seattle 98195, USA.
Olney
R K
RK
D000200:Action Potentials; D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology* / Q000503:physiopathology; D002452:Cell Count; D018450:Disease Progression; D005260:Female; D018737:Hand Strength; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000473:pathology*; D018485:Muscle Fibers, Skeletal / Q000648:ultrastructure*; D009132:Muscles / Q000503:physiopathology; D013997:Time Factors