Amyotrophic lateral sclerosis (ALS) is a late-onset fatal neurodegenerative disease affecting
motor neurons with an incidence of about 1/100,000. Most ALS cases are sporadic, but 5 …

Background Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease, is a
fatal neurodegenerative disease. Neuromuscular respiratory failure is the most common …

Background Few patients with amyotrophic lateral sclerosis currently receive non-invasive
ventilation (NIV), reflecting clinical uncertainty about the role of this intervention. We aimed …

Although ventilatory failure is the most common cause of death in amyotrophic lateral
sclerosis (ALS) and measurement of respiratory muscle strength (RMS) has been shown to …

Background: Noninvasive ventilation (NIV) probably improves survival in ALS, but the
magnitude and duration of any improvement in quality of life (QoL) and the optimal criteria …

The management of motor neurone disease (MND) has evolved rapidly over the last two
decades. Although still incurable, MND is not untreatable. From an attitude of nihilism …

Amyotrophic lateral sclerosis (ALS) exhibits characteristic variability of onset and rate of
disease progression, with inherent clinical heterogeneity making disease quantitation …

Rationale: Duchenne muscular dystrophy (DMD) leads to progressive, generalized paresis,
and to respiratory failure in the second decade of life. The assumption that severe physical …

Respiratory muscle weakness is the usual cause of death in amyotrophic lateral sclerosis.
The prognostic value of the forced vital capacity (FVC), mouth-inspiratory force, and sniff …

Respiratory and skeletal muscle function is of interest in many areas of pulmonary and
critical care medicine. The capacity of the respiratory muscle pump to respond to the load …