Nocturnal pulse oximetry: a new approach to establish the appropriate time for non-invasive ventilation in ALS patients.

Previous studies have supported non-invasive ventilation (NIV) in amyotrophic lateral sclerosis (ALS), indicating that it prolongs survival. However, criteria for the use of NIV are yet to be defined. The aim of the present study was to evaluate the potential use of nocturnal pulse oximetry (NPO) as a tool for determining the most appropriate time at which to initiate NIV in ALS patients.

Allgrove syndrome in adulthood.

A 35-year-old man with a past history of achalasia developed progressive spastic tetraparesis, distal limb atrophy, dysarthria, and dysphagia. A clinical diagnosis of amyotrophic lateral sclerosis (ALS) was considered before neurophysiological investigation, which disclosed a polyneuropathy and a prolonged central conduction time. One year later, the patient developed dysautonomic symptoms.

Respiratory disorders in ALS: sleep and exercise studies.

Sleep disruption in ALS/MND is related to hypoventilation and nocturnal O(2) saturation. Maximal inspiratory pressure (PI(max)) proved sensitive in predicting nocturnal O(2) saturation. However, PI(max) is highly dependent on patient collaboration; on the other hand Mouth Occlusion Pressure (MOP) is a reliable, non-volitional parameter index of central respiratory drive.