Single fibre electromyography in the extensor digitorum communis muscle was studied in five patients with central core disease. The average number of muscle fibre action potentials belonging to the same motor unit was higher in patients than in healthy subjects of the same age. The increase in motor unit fibre density is consistent with increased terminal innervation ratio described in other pa...

Dystrophic muscle shows increase in fibre density, abnormally low jitter in some recordings and more often increased jitter. The cross section of the motor unit has normal length. There are no signs of abnormal volume conduction characteristics. The increased fibre density is believed to be due to localised increase in the number of muscle action potential generators. The findings are compatibl...

Motor unit number estimation (MUNE) is a method which aims to provide a quantitative indicator of progression of diseases that lead to loss of motor units, such as motor neurone disease. However the development of a reliable, repeatable and fast real-time MUNE method has proved elusive hitherto. Ridall et al. (2007) implement a reversible jump Markov chain Monte Carlo (RJMCMC) algorithm to prod...

Age-related changes in the basic functional unit of the neuromuscular system, the motor unit, and its neural inputs have a profound effect on motor function, especially among the expanding number of old (older than ∼60 yr) and very old (older than ∼80 yr) adults. This review presents evidence that age-related changes in motor unit morphology and properties lead to impaired motor performance tha...

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease which attacks the motor system. Current treatment for sporadic ALS or Cu, Zn superoxide dimutase 1 (SOD 1 mutation) familial ALS, produces only a modest increase in survival. Riluzole, has been available since 1995 and remains the only disease modifying therapy available for ALS. Using the statistical motor unit number est...