文档介绍：Andrea Vianello
Fisiopatologia e Terapia Intensiva Respiratoria
Ospedale-Università di Padova
Lo Pneumologo
Symptoms of ALS
First signs and symptoms (frequently overlooked)
Twitching and cramping of muscles (especially in hands and feet)
Stiffness
Weakness (especially in hands, arms and legs)
Slurred speech
Picture taken from the National Institute of Aging
Symptoms continued . . .
Later signs and symptoms:
Difficulty chewing and swallowing
Shortness of breath
Muscle weakness due to wasting away of muscles
Causes muscles to e smaller
Respiratory failure
Paralysis
Picture from the ALS Association
Clinical and Pulmonary Function evaluation
ALS
NMD
Respir Care 2013;58:1433-41
a & Vianello
abdomen
Chest wall
abdomen
Chest wall
Chest wall
diaphragm
abdomen
diaphragm
diaphragm
Normal subject
Diaphragmatic fatigue
Intercostal muscle fatigue
Cause of death in 302 French patients with ALS
Most patients (63%) died in a medical facility
The most frequently reported cause of death was:
respiratory failure (77%) including:
terminal respiratory insufficiency (58%)
pneumonia (14%)
asphyxia due to a foreign body (3%)
pulmonary embolism (2%)
Ten per cent of patients died from other causes: post-surgical or traumatic conditions (5%), cardiac causes (%), suicide (%) and sudden death of unknown origin (%)
The cause of death could not be determined in 13% of cases (6% inside a medical facility and 25% outside)
Gil J, Funalot B, Verschueren A et al. Causes of death amongst French patients with amyotrophic lateral sclerosis: a prospective study. Eur J Neurol 2008;15:1245–51.
Long-Term Noninvasive Ventilation
What is Noninvasive Ventilation?
“A form of ventilatory support that avoids airway invasion”