Skip to main content
AAN.com
Articles
December 11, 2001

Respiratory function vs sleep-disordered breathing as predictors of QOL in ALS

December 1, 2001 issue
57 (11) 2040-2044

Abstract

Background: Most patients with ALS have evidence of respiratory muscle weakness at diagnosis, and death is usually due to respiratory failure. Sleep disruption, possibly due to apneas, hypopneas, orthopnea, or REM-related desaturation, is common. The relative impact of these factors on quality of life has not been established.
Methods: The authors recruited 23 subjects with probable or definite ALS. Quality of life was assessed using generic and specific instruments, and respiratory muscle strength by measurement of vital capacity, maximum static pressures, and sniff nasal inspiratory pressure. Twenty-two subjects underwent polysomnography. Overall limb and axial muscle strength was estimated using a summated muscle score based on the Medical Research Council clinical scale.
Results: On univariate analysis, there were moderate to strong correlations between quality of life and all measurements of respiratory muscle function (R = 0.42–0.82). The correlations with selected polysomnographic indices were weaker and less consistent (R = 0.44–0.59). Multivariate analysis showed that maximum static inspiratory pressure was the strongest independent predictor of quality of life.
Conclusion: Quality of life was strongly and independently related to respiratory muscle function. Relations with polysomnographic indices were weaker and were attributable to respiratory muscle weakness. Respiratory muscle weakness is much more important than the frequency of apneas and hypopneas in determining quality of life in ALS.

Get full access to this article

View all available purchase options and get full access to this article.

References

1.
Schiffman PL, Belsh JM. Pulmonary function at diagnosis of amyotrophic lateral sclerosis. Rate of deterioration. Chest . 1993; 103: 508–513.
2.
Fallat RJ, Jewitt B, Bass M, Kamm B, Norris FH, Jr. Spirometry in amyotrophic lateral sclerosis. Arch Neurol 1979;36:74–80.
3.
Fitting J-W, Paillex R, Hirt L, Aebischer P, Schluep M. Sniff nasal pressure: a sensitive respiratory test to assess progression of amyotrophic lateral sclerosis. Ann Neurol . 1999; 46: 887–893.
4.
Gay PC, Westbrook PR, Daube JR, Litchy WJ, Windebank AJ, Iverson R. Effects of alterations in pulmonary function and sleep variables on survival in patients with amyotrophic lateral sclerosis. Mayo Clin Proc . 1991; 66: 686–694.
5.
Ferguson KA, Strong MJ, Ahmad D, George CF. Sleep-disordered breathing in amyotrophic lateral sclerosis. Chest . 1996; 110: 664–669.
6.
David WS, Bundlie SR, Mahdavi Z. Polysomnographic studies in amyotrophic lateral sclerosis. J Neurol Sci . 1997; 152 (suppl 1): S29–35.
7.
Quanjer PH. Standardised lung function testing. Report working party. Standardisation of lung function tests. European community for coal and steel. Bull Eur Physiopathol Respir . 1983; 19 (suppl 5): 1–82.
8.
Wilson SH, Cooke NT, Edwards RHT, Spiro SG. Predicted normal values for maximal respiratory pressures in Caucasian adults and children. Thorax . 1984; 39: 535–538.
9.
Uldry C, Fitting JW. Maximal values of sniff nasal inspiratory pressure in healthy subjects. Thorax . 1995; 50: 371–375.
10.
Bensimon G, Lacomblez L, Meninger V. A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group. N Engl J Med . 1994; 330: 585–591.
11.
Rechtschaffen A, Kales A. A manual of standardised terminology, techniques and scoring system for sleep stages of human subjects. Los Angeles: UCLA Brain Information Service/Brain Research Institute, 1968: 1–12.
12.
American Sleep Disorders Association. EEG arousals: scoring rules and examples. A preliminary report from the Sleep Disorders Atlas Task Force of the American Sleep Disorders Association. Sleep . 1992; 15: 174–184.
13.
American Academy of Sleep Medicine Task Force. Sleep-related breathing disorders in adults. Recommendations for syndrome definition and measurement techniques in clinical research. Sleep . 1999; 22: 667–689.
14.
Jenkinson C, Layte R, Wright L, Coulter A. The UK SF-36: an analysis and interpretation manual. Oxford: Health Services Research Unit, University of Oxford, 1996.
15.
Dupuy HJ. Assessment of quality of life in clinical trials for cardiovascular therapies. New York: LeJacq, 1984.
16.
Shields RK, Ruhland JL, Ross MA, Saehler MM, Smith KB, Heffner ML. Analysis of health-related quality of life and muscle impairment in individuals with amyotrophic lateral sclerosis using the medical outcome survey and the Tufts Quantitative Neuromuscular Exam. Arch Phys Med Rehabil . 1998; 79: 855–862.
17.
Guyatt GH, Berman LB, Townsend M, Pugsley SO, Chambers LW. A measure of quality of life for clinical trials in chronic lung disease. Thorax . 1987; 42: 773–778.
18.
Crockett AJ, Cranston JM, Moss JR, Alpers JH. Effects of long-term oxygen therapy on quality of life and survival in chronic airflow limitation. Monaldi Arch Chest Dis . 1999; 54: 193–196.
19.
Flemons WW, Reimer MA. Development of a disease-specific health-related quality of life questionnaire for sleep apnea. Am J Respir Crit Care Med . 1998; 158: 494–503.
20.
Johns MW. Sleepiness in different situations measured by the Epworth Sleepiness Scale. Sleep . 1994; 17: 703–710.
21.
Cedarbaum JM, Stambler N. Performance of the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) in multicenter trials. J Neurol Sci . 1997; 152 (suppl 1): S1–9.
22.
Williams RL, Karacan I, Hursch CJ. Electroencephalography (EEG) of human sleep: Clinical applications. New York: Wiley, 1974.
23.
Kimura K, Tachibana N, Kimura J, Shibasaki H. Sleep-disordered breathing at an early stage of amyotrophic lateral sclerosis. J Neurol Sci . 1999; 164: 37–43.
24.
Arnulf I, Similowski T, Salachas F, et al. Sleep disorders and diaphragmatic function in patients with amyotrophic lateral sclerosis. Am J Respir Crit Care Med . 2000; 161: 849–856.
25.
Miller RG, Munsat TL, Swash M, Brooks BR. Consensus guidelines for the design and implementation of clinical trials in ALS. World Federation of Neurology Committee on Research. J Neurol Sci . 1999; 169: 2–12.
26.
Barbe F, Quera-Salva MA, McCann C, et al. Sleep-related respiratory disturbances in patients with Duchenne muscular dystrophy. Eur Respir J . 1994; 7: 1403–1408.
27.
Pinto AC, Evangelista T, Carvalho M, Alves MA, Sales Luis ML. Respiratory assistance with a non-invasive ventilator (Bipap) in MND/ALS patients: survival rates in a controlled trial. J Neurol Sci . 1995; 129 (suppl): 19–26.
28.
Kleopa KA, Sherman M, Neal B, Romano GJ, Heiman-Patterson T. Bipap improves survival and rate of pulmonary function decline in patients with ALS. J Neurol Sci . 1999; 164: 82–88.

Information & Authors

Information

Published In

Neurology®
Volume 57Number 11December 1, 2001
Pages: 2040-2044
PubMed: 11739823

Publication History

Received: April 4, 2001
Accepted: August 29, 2001
Published in print: December 1, 2001
Published online: December 11, 2001

Permissions

Request permissions for this article.

Authors

Affiliations & Disclosures

S.C. Bourke, MB, Bch MRCP
From the University of Newcastle (Dr. Bourke and Prof. Gibson), Newcastle upon Tyne; University of Sheffield and Royal Hallamshire Hospital (Prof. Shaw), Sheffield; and Freeman Hospital (Prof. Gibson), Newcastle upon Tyne, UK.
P.J. Shaw, MD, FRCP
From the University of Newcastle (Dr. Bourke and Prof. Gibson), Newcastle upon Tyne; University of Sheffield and Royal Hallamshire Hospital (Prof. Shaw), Sheffield; and Freeman Hospital (Prof. Gibson), Newcastle upon Tyne, UK.
G.J. Gibson, MD, FRCP
From the University of Newcastle (Dr. Bourke and Prof. Gibson), Newcastle upon Tyne; University of Sheffield and Royal Hallamshire Hospital (Prof. Shaw), Sheffield; and Freeman Hospital (Prof. Gibson), Newcastle upon Tyne, UK.

Notes

Address correspondence and reprint requests to Dr. S.C. Bourke, Sir William Leech Centre for Lung Research, Freeman Hospital, Newcastle upon Tyne, NE7 7DN, UK; e-mail: [email protected]

Metrics & Citations

Metrics

Citation information is sourced from Crossref Cited-by service.

Citations

Download Citations

If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Select your manager software from the list below and click Download.

Cited By
  1. A qualitative exploratory study into medical, nursing and allied health professional experiences of elective withdrawal of non‐invasive ventilation in a motor neurone disease cohort, Journal of Evaluation in Clinical Practice, (2024).https://doi.org/10.1111/jep.14166
    Crossref
  2. The glymphatic system and Amyotrophic lateral sclerosis, Progress in Neurobiology, 234, (102571), (2024).https://doi.org/10.1016/j.pneurobio.2024.102571
    Crossref
  3. Management of motor neuron disease with updated therapies: A review, IP Indian Journal of Neurosciences, 9, 1, (1-7), (2023).https://doi.org/10.18231/j.ijn.2023.001
    Crossref
  4. Exploring inspiratory occlusion metrics to assess respiratory drive in patients under acute intermittent hypoxia, Respiratory Physiology & Neurobiology, 304, (103922), (2022).https://doi.org/10.1016/j.resp.2022.103922
    Crossref
  5. Nonsteroidal anti-inflammatory drug (ketoprofen) delivery differentially impacts phrenic long-term facilitation in rats with motor neuron death induced by intrapleural CTB-SAP injections, Experimental Neurology, 347, (113892), (2022).https://doi.org/10.1016/j.expneurol.2021.113892
    Crossref
  6. How Are Adenosine and Adenosine A2A Receptors Involved in the Pathophysiology of Amyotrophic Lateral Sclerosis?, Biomedicines, 9, 8, (1027), (2021).https://doi.org/10.3390/biomedicines9081027
    Crossref
  7. Medical therapies for amyotrophic lateral sclerosis-related respiratory decline: an appraisal of needs, opportunities and obstacles, Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 23, 1-2, (66-75), (2021).https://doi.org/10.1080/21678421.2021.1920981
    Crossref
  8. An Introduction to Advanced Lung Disease, Palliative Care in Lung Disease, (11-25), (2021).https://doi.org/10.1007/978-3-030-81788-6_2
    Crossref
  9. Diaphragmatic dysfunction at the first visit to a chest diseases outpatient clinic in 500 patients with amyotrophic lateral sclerosis, Muscle & Nerve, 63, 5, (683-689), (2021).https://doi.org/10.1002/mus.27200
    Crossref
  10. Associations of Patient Mood, Modulators of Quality of Life, and Pharmaceuticals with Amyotrophic Lateral Sclerosis Survival Duration, Behavioral Sciences, 10, 1, (33), (2020).https://doi.org/10.3390/bs10010033
    Crossref
  11. See more
Loading...

View Options

Login options

Check if you have access through your login credentials or your institution to get full access on this article.

Personal login Institutional Login
Purchase Options

The neurology.org payment platform is currently offline. Our technical team is working as quickly as possible to restore service.

If you need immediate support or to place an order, please call or email customer service:

  • 1-800-638-3030 for U.S. customers - 8:30 - 7 pm ET (M-F)
  • 1-301-223-2300 for customers outside the U.S. - 8:30 - 7 pm ET (M-F)
  • [email protected]

We appreciate your patience during this time and apologize for any inconvenience.

View options

PDF and All Supplements

Download PDF and Supplementary Material

Full Text

View Full Text

Full Text HTML

View Full Text HTML

Media

Figures

Other

Tables

Share

Share

Share article link

Share