Amyotrophic Lateral Sclerosis and the Respiratory System

Andrew T Braun; Candelaria Caballero-Eraso; Noah Lechtzin

doi:10.1016/j.ccm.2018.01.003

Amyotrophic Lateral Sclerosis and the Respiratory System

Clin Chest Med. 2018 Jun;39(2):391-400. doi: 10.1016/j.ccm.2018.01.003.

Authors

Andrew T Braun¹, Candelaria Caballero-Eraso², Noah Lechtzin³

Affiliations

¹ 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; Division of Allergy, Pulmonary, and Critical Care, Department of Medicine, University of Wisconsin School of Medicine and Public Health, 600 Highland Avenue, Madison, WI 53792, USA.
² 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; Medical-Surgical Unit of Respiratory Diseases, Institute of Biomedicine of Seville (IBiS), Centre for Biomedical Research in Respiratory Diseases Network (CIBERES), University Hospital Virgen del Rocío, University of Seville, Avenida Dr. Fedriani, 41009 Sevilla, Spain.
³ 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: nlechtz@jhmi.edu.

PMID: 29779597
DOI: 10.1016/j.ccm.2018.01.003

Abstract

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.

Keywords: Diaphragm; Noninvasive ventilation; Secretion clearance.

Publication types

Review

MeSH terms

Amyotrophic Lateral Sclerosis / physiopathology*
Humans