Polysomnography-directed Noninvasive Ventilation in Amyotrophic Lateral Sclerosis (ALS)

Progression of Respiratory Dysfunction in Amyotrophic Lateral Sclerosis (ALS) Patients: A Comparison of Standard of Practice vs Polysomnography-Directed Nocturnal Non-Invasive Positive Pressure Ventilation

Use of noninvasive ventilation (NIV, also known colloquially as "Bipap") has been associated in some studies with improvement in pulmonary function, quality of life and survival. NIV is typically applied during sleep, and without the benefit of sleep study to determine the optimal settings. The investigators have shown that when NIV is used in this fashion, failure of nocturnal oxygenation and ventilation is prominent. This study is randomizing patients to standard application of NIV vs application guided by use of sleep study data to determine the effect of titrated therapy on pulmonary function, quality of life and survival.

Inclusion Criteria:

• Diagnosis of probable or definite Amyotrophic Lateral Sclerosis (ALS) per El Escorial criteria
• Between ages of 18 and 80 yrs old

Exclusion Criteria:

• Inability to clear secretions from the airway
• Life expectancy < 6 months from a comorbid condition
• Dementia sufficient to impair ability to use NIV, perform respiratory muscle pressure testing (PFTs), or complete Health-related Quality of Life (HRQOL) instruments
• Inability to follow up at the ALS Center on a regular basis
• Previously diagnosed obstructive Sleep Apnea