title: Motoneuron firing in amyotrophic lateral sclerosis (ALS).
abstract: Amyotrophic lateral sclerosis is an inexorably progressive neurodegenerative disorder involving the classical motor system and the frontal effector brain, causing muscular weakness and atrophy, with variable upper motor neuron signs and often an associated fronto-temporal dementia. The physiological disturbance consequent on the motor system degeneration is beginning to be well understood. In this review we describe aspects of the motor cortical, neuronal, and lower motor neuron dysfunction. We show how studies of the changes in the pattern of motor unit firing help delineate the underlying pathophysiological disturbance as the disease progresses. Such studies are beginning to illuminate the underlying disordered pathophysiological processes in the disease, and are important in designing new approaches to therapy and especially for clinical trials.
authors.affiliation: Institute of Physiology and Institute of Molecular Medicine, Faculty of Medicine, University of Lisbon Lisbon, Portugal ; Department of Neurosciences, Hospital Santa Maria, Faculty of Medicine, University of Lisbon Lisbon, Portugal.
authors.forename: Mamede
authors.initials: M
authors.lastname: de Carvalho
authors.affiliation: Emeritus Professor of Neurology, University of British Columbia Vancouver, BC, Canada.
authors.forename: Andrew
authors.initials: A
authors.lastname: Eisen
authors.affiliation: Department of Biomedical Physiology and Kinesiology, Simon Fraser University, Burnaby BC, Canada ; Department of Medicine (Neurology), University of British Columbia, Vancouver BC, Canada.
authors.forename: Charles
authors.initials: C
authors.lastname: Krieger
authors.affiliation: Institute of Physiology and Institute of Molecular Medicine, Faculty of Medicine, University of Lisbon Lisbon, Portugal ; Department of Neurosciences, Hospital Santa Maria, Faculty of Medicine, University of Lisbon Lisbon, Portugal ; Institute of Neuroscience, Barts and The London School of Medicine, Queen Mary University of London London, UK.
authors.forename: Michael
authors.initials: M
authors.lastname: Swash
keywords: amyotrophic lateral sclerosis; lower motor neuron; motor cortex; motor units firing; upper motor neuron