Noninvasive ventilation in amyotrophic lateral sclerosis: effects on sleep quality and quality of life.
OBJECTIVE
Little is known about the effects of noninvasive ventilation (NIV) on sleep quality in amyotrophic lateral sclerosis (ALS). We aim to evaluate the long-term effects of NIV on sleep quality and quality of life in patients with ALS.


METHODS
In this prospective observational study, 13 ALS patients were followed for one year after initiating NIV. We evaluated sleep quality, quality of life and functional status with several questionnaires: Epworth sleepiness Scale (ESS), Pittsburg sleep quality index (PSQI), Short Form 36 Health Questionnaire (SF-36), McGill Quality of Life questionnaire (McGillQoL) and revised Amyotrophic Lateral Sclerosis Functional Rating Scale scores (ALSFRS-R).


RESULTS
Median and interquartile range (IQR) at the start of NIV was 59 (53-65) years. The ALSFRS-R at start was 30 (24-37) (median, IQR), with three patients having severe bulbar impairment (ALSFRS-R-bulbar ≤ 9). The PaCO2at start of NIV treatment was 48 (43-52) mmHg (median, IQR). During the one-year follow-up period, a significant decrease in the ALSFRS-R was observed. The impact of NIV in a short term (1 month) revealed a statistically significant decrease in ESS, decrease in total PSQI and of four PSQI subscales and improvement of almost all subscales of the McGill questionnaire. Long-term analyses (9 months to 1 year) revealed that amelioration in ESS and total PSQI was sustained.


CONCLUSIONS
We conclude that accurately titrated NIV in ALS patients can stabilize sleep quality and quality of life for at least one year, despite significant disease progression.
a Department of Pulmonology , University of Leuven , Leuven , Belgium.
Eva
E
Vandoorne
a Department of Pulmonology , University of Leuven , Leuven , Belgium.|b Leuven University Centre for Sleep/Wake Disorders, Department of Pulmonology , University Hospitals Leuven , Leuven , Belgium.
Bart
B
Vrijsen
a Department of Pulmonology , University of Leuven , Leuven , Belgium.|b Leuven University Centre for Sleep/Wake Disorders, Department of Pulmonology , University Hospitals Leuven , Leuven , Belgium.
Catharina
C
Belge
a Department of Pulmonology , University of Leuven , Leuven , Belgium.|b Leuven University Centre for Sleep/Wake Disorders, Department of Pulmonology , University Hospitals Leuven , Leuven , Belgium.
Dries
D
Testelmans
a Department of Pulmonology , University of Leuven , Leuven , Belgium.|b Leuven University Centre for Sleep/Wake Disorders, Department of Pulmonology , University Hospitals Leuven , Leuven , Belgium.
Bertien
B
Buyse
D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D063087:Noninvasive Ventilation / Q000379:methods*; D017286:Polysomnography; D011446:Prospective Studies; D011788:Quality of Life; D012720:Severity of Illness Index; D012890:Sleep / Q000502:physiology*; D012893:Sleep Wake Disorders / Q000209:etiology / Q000503:physiopathology*; D011795:Surveys and Questionnaires; D013997:Time Factors
Amyotrophic lateral sclerosis; Noninvasive; Quality of life; Sleep; Ventilation