Reliability of autonomic activations as surrogates of cortical arousals in ventilated patients affected by amyotrophic lateral sclerosis

Purpose. The study aims to evaluate the performance of autonomic activations as a tool to assess sleep fragmentation and to recognize hypopneas in patients with amyotrophic lateral sclerosis (ALS) under non-invasive mechanical ventilation and secondarily, to evaluate, in patients with the same disease, the relationship between disruption of autonomic nervous system (ANS) activity and the usefulness of the autonomic activations as surrogates of cortical arousals. Methods. Sixteen ALS patients underwent simultaneous polysomnography and portable cardiorespiratory monitoring (PM). On the polysomnography, standard rules were used for scoring arousals and respiratory events. On the PM, autonomic arousals were scored as >= 15% heart rate (HR) increase with a >= 35% pulse wave amplitude (PWA) reduction, HR increase >= 20%, or PWA decrease >= 40%. Nocturnal HR variability was analyzed in the ALS patients and in 11 control subjects as an index of ANS activity. Results. Synchronized epoch by epoch analysis of the polysomnography and PMrecordings showed that only 31.0 (22.5-58.7)% cortical and 36.1 (20.5-47.2)% autonomic arousals were associated with one another. Among hypopneas scored at polysomnography, 71.7% were associated with a cortical arousal but not with a desaturation. On average, HR variability in ALS showed signs of depressed ANS activity that was particularly evident in the patients where the cortical arousals exceeded the autonomic ones. Conclusions. In ventilated ALS patients, autonomic activations may hardly have a role as surrogates of cortical arousals for assessment of sleep fragmentation and for respiratory scoring. Depression of ANS activity may be related to their poor performance.