title: Ventilatory control in ALS.
abstract: Amyotrophic lateral sclerosis (ALS) is a fatal, progressive neurodegenerative disease. ALS selectively causes degeneration in upper and lower (spinal) motor neurons, leading to muscle weakness, paralysis and death by ventilatory failure. Although ventilatory failure is generally the cause of death in ALS, little is known concerning the impact of this disorder on respiratory motor neurons, the consequences of respiratory motor neuron cell death, or the ability of the respiratory control system to "fight back" via mechanisms of compensatory respiratory plasticity. Here we review known effects of ALS on breathing, including possible effects on rhythm generation, respiratory motor neurons, and their target organs: the respiratory muscles. We consider evidence for spontaneous compensatory plasticity, preserving breathing well into disease progression despite dramatic loss of spinal respiratory motor neurons. Finally, we review current and potential therapeutic approaches directed toward preserving the capacity to breathe in ALS patients.
authors.affiliation: Department of Comparative Biosciences, University of Wisconsin, School of Veterinary Medicine, 2015 Linden Drive, Madison, WI 53706, USA.
authors.forename: Nicole L
authors.initials: NL
authors.lastname: Nichols
authors.forename: J
authors.initials: J
authors.lastname: Van Dyke
authors.forename: L
authors.initials: L
authors.lastname: Nashold
authors.forename: I
authors.initials: I
authors.lastname: Satriotomo
authors.forename: M
authors.initials: M
authors.lastname: Suzuki
authors.forename: G S
authors.initials: GS
authors.lastname: Mitchell
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy*; D000818:Animals; D006801:Humans; D007385:Intermittent Positive-Pressure Ventilation / Q000379:methods; D009415:Nerve Net / Q000502:physiology; D063087:Noninvasive Ventilation / Q000379:methods*; D012123:Pulmonary Ventilation / Q000502:physiology*; D012125:Respiratory Center / Q000502:physiology*
keywords: Breathing; Compensation; Motor neuron disease; Neurodegenerative disease; Plasticity; Ventilatory control