Treatment strategies in amyotrophic lateral sclerosis and its mimics

Background: Symptoms of nocturnal hypoventilation may negatively influence the quality of life (QoL) of ALS patients before respiratory failure ensues. Non-invasive mechanical ventilation (NIV) is considered a treatment option for nocturnal hypoventilation. The primary objective of NIV is improving quality of life (QoL). It may also prolong life with several months. Objective: A systematic review of the literature was performed to analyze what is known of the effect of NIV on survival, QoL and other outcome measures. Methods: A computerized literature search was performed to identify controlled clinical trials and observational studies of treatment of ALS – associated nocturnal hypoventilation from 1985 until May 2005. Results: Twelve studies fulfilled the inclusion criteria. Four studies were retrospective, 7 prospective and in one study randomization was used. All studies reported beneficial effects of NIV on all outcome measures. In 7 studies NIV was associated with prolonged survival in patients tolerant for NIV, 5 studies reported an improved QoL. Conclusion: Studies on the use of NIV in ALS differ in study design and endpoint definitions. All studies suggest a beneficial effect on QoL and other outcome measures (Evidence level Class II-III). Well-designed randomized controlled trials comparing the effect on QoL and survival have not been performed. 30 Chapter 2.1 Effects of non-invasive ventilation in ALS