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Breathing new life into treatment advances for respiratory failure in amyotrophic lateral sclerosis patients
Practice Points
Susana Pinto*1 & Mamede de Carvalho1,2 Respiratory evaluation should be performed in all amyotrophic lateral sclerosis (ALS) patients at the first
visit at the tertiary referral hospital and every 3 months thereafter. Respiratory evaluation in ALS patients should include standardized clinical evaluation plus respiratory tests. The most critical clinical respiratory symptoms and signs are: dyspnea for light efforts and at rest; use
of respiratory accessory muscles and tachypnea at rest; paradoxical respiration; orthopnea; poor sleep efficacy; morning headache; daily fatigue or daily sleepiness; and ineffective cough. The most relevant respiratory function tests are: forced vital capacity; forced expiratory flow; peak cough
expiratory flow; maximal inspiratory pressure; maximal expiratory pressure; nasal inspiratory pressure during a sniff; blood gases; and nocturnal pulse oxymetry or polyssonography. Maximal voluntary ventilation and phrenic nerve studies should also be considered. According to European guidelines, noninvasive ventilation (NIV) should be considered and discussed
with ALS patients and caregivers when there is at least one respiratory insufficiency symptom and at least one objective parameter confirming respiratory insufficiency (forced vital capacity:
Breathing new life into treatment advances for respiratory failure in amyotrophic lateral sclerosis patients
Practice Points
Susana Pinto*1 & Mamede de Carvalho1,2 Respiratory evaluation should be performed in all amyotrophic lateral sclerosis (ALS) patients at the first
visit at the tertiary referral hospital and every 3 months thereafter. Respiratory evaluation in ALS patients should include standardized clinical evaluation plus respiratory tests. The most critical clinical respiratory symptoms and signs are: dyspnea for light efforts and at rest; use
of respiratory accessory muscles and tachypnea at rest; paradoxical respiration; orthopnea; poor sleep efficacy; morning headache; daily fatigue or daily sleepiness; and ineffective cough. The most relevant respiratory function tests are: forced vital capacity; forced expiratory flow; peak cough
expiratory flow; maximal inspiratory pressure; maximal expiratory pressure; nasal inspiratory pressure during a sniff; blood gases; and nocturnal pulse oxymetry or polyssonography. Maximal voluntary ventilation and phrenic nerve studies should also be considered. According to European guidelines, noninvasive ventilation (NIV) should be considered and discussed
with ALS patients and caregivers when there is at least one respiratory insufficiency symptom and at least one objective parameter confirming respiratory insufficiency (forced vital capacity:
