Mutations of Cu,Zn superoxide dismutase cause an autosomal dominant form of familial amyotrophic lateral sclerosis. An animal model of the disease has been produced by expressing mutant human SOD1 in transgenic mice (G93A). In order to quantify the dysfunction of the motor unit in transgenic mice, e
Functional significance of upper and lower motor neuron impairment in amyotrophic lateral sclerosis
โ Scribed by Jane A. Kent-Braun; Christie H. Walker; Michael W. Weiner; Robert G. Miller
- Publisher
- John Wiley and Sons
- Year
- 1998
- Tongue
- English
- Weight
- 132 KB
- Volume
- 21
- Category
- Article
- ISSN
- 0148-639X
No coin nor oath required. For personal study only.
โฆ Synopsis
The objective of this study was to examine the contribution of lower motor neuron (LMN) and upper motor neuron (UMN) dysfunction to weakness and impaired motor control in 27 patients with amyotrophic lateral sclerosis (ALS). Isometric strength was measured by dorsiflexor maximum voluntary contraction force (MVC). LMN function was measured by tetanic force, twitch force, and the amplitude of the compound muscle action potential. UMN function was measured by the speed of rapid foot taps, the maximum rate of rise of voluntary force, and the central activation ratio [CAR = MVC/(MVC + superimposed tetanic force)]. The results suggest that (1) LMN loss appears to be the primary cause of progressive weakness in ALS; while (2) UMN impairment in ALS leads to slowing of contraction speed and rapid movements, and modest decreases in central activation; and (3) during 6 months of progression, LMN changes were greater than UMN changes.
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