Amyotrophic Lateral Sclerosis and the Respiratory System.

Clin Chest Med

Division of Pulmonary and Critical Care and Sleep Medicine, Department of Medicine, Johns Hopkins University School of Medicine, 1830 East Monument Street, Baltimore, MD 21205, USA. Electronic address:

Published: June 2018

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that always affects the respiratory muscles. It is characterized by degeneration of motor neurons in the brain and spinal cord. Respiratory complications are the most common causes of death in ALS and typically occur within 3 to 5 years of diagnosis. Because ALS affects both upper and lower motor neurons, it causes hyperreflexia, spasticity, muscle fasciculations, muscle atrophy, and weakness. It ultimately progresses to functional quadriplegia. ALS most commonly begins in the limbs, but in about one-third of cases it begins in the bulbar muscles responsible for speech and swallowing.

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Source
http://dx.doi.org/10.1016/j.ccm.2018.01.003DOI Listing

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