Purpose: Can early diaphragmatic pacing in ALS delay the need for noninvasive ventilation?
Background: ALS is a neurodegenerative disease associated with weakness of the diaphragm and respiratory insufficiency. Non-invasive ventilation relieves respiratory distress and prolongs survival. Early diaphragmatic stimulation was hoped to delay diaphragmatic weakening even before respiratory insufficiency.
PATIENTS AND METHODS: The RespiStimALS is a triple-blinded randomized controlled trial in patients with definite or probable ALS in France. Inclusion criteria included moderate respiratory distress (expected forced vital capacity 60-80%) and bilateral diaphragmatic response to diagnostic phrenic nerve stimulation. All patients received a phrenic stimulator laparoscopically, and either an active or an inactive sham stimulator was randomly assigned (1:1). The primary endpoint was survival to the need for noninvasive ventilation.
Results: Between 2012 and 2015, 74 participants were included. After a study (DiPALS) in England showed increased mortality in respiratory failure ALS patients under diaphragmatic pacing, an unplanned analysis was performed in 2015 with also increased mortality in the “active” group. In addition, median survival to the need for non-invasive ventilation was 6.0 in the “active” group, 8.8 months in the control group (p=0.02).
Authors’ conclusions: Early diaphragmatic pacing in ALS patients does not delay the need for noninvasive ventilation, has no benefit on quality of life, and actually decreases survival. The reason for the harmful effect is not yet clear.
InFo NEUROLOGY & PSYCHIATRY 2017; 15(2): 27.
