Amyotrophic Lateral Sclerosis and the Respiratory System - 18/05/18

Doi : 10.1016/j.ccm.2018.01.003

Andrew T. Braun, MD, MHS ^a,^b, Candelaria Caballero-Eraso, MD, PhD ^a,^c, Noah Lechtzin, MD, MHS ^a,^⁎
^a 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
^b 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
^c 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

^∗Corresponding author.1830 East Monument Street5th FloorBaltimoreMD21205

Résumé

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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Keywords : Noninvasive ventilation, Diaphragm, Secretion clearance

Plan

Key points

Introduction

Respiratory manifestations

Inspiratory Muscle Weakness

Expiratory Muscle Weakness

Bulbar-Innervated Muscles Dysfunction

Respiratory assessment in amyotrophic lateral sclerosis

Pulmonary Function Testing

Nocturnal Oximetry and Polysomnography

Treatment of respiratory complications in amyotrophic lateral sclerosis

Improving Ventilation

Secretion management

Tracheostomy/Mechanical Ventilation and End-of-Life Care

Palliative care

Withdrawal of life support

Summary

Disclosures: Dr N. Lechtzin has served as a consultant with Hill-Rom, Inc, Dr A. Braun and Dr C. Caballero-Eraso have no relevant conflicts of interest or financial disclosures.