Volume 63, Issue 5 pp. 683-689

CLINICAL RESEARCH ARTICLE

Diaphragmatic dysfunction at the first visit to a chest diseases outpatient clinic in 500 patients with amyotrophic lateral sclerosis

Aylin Pihtili MD,

Aylin Pihtili MD

Istanbul Faculty of Medicine, Department of Pulmonary Medicine, Istanbul University, Istanbul, Turkey

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Zuleyha Bingol MD,

Zuleyha Bingol MD

Istanbul Faculty of Medicine, Department of Pulmonary Medicine, Istanbul University, Istanbul, Turkey

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Hacer Durmus MD,

Hacer Durmus MD

Istanbul Faculty of Medicine, Department of Neurology, Istanbul University, Istanbul, Turkey

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Yesim Parman MD,

Yesim Parman MD

Istanbul Faculty of Medicine, Department of Neurology, Istanbul University, Istanbul, Turkey

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Esen Kiyan MD,

Corresponding Author

Esen Kiyan MD

[email protected]

Istanbul Faculty of Medicine, Department of Pulmonary Medicine, Istanbul University, Istanbul, Turkey

Correspondence

Esen Kiyan, Istanbul University, Istanbul Faculty of Medicine, Department of Pulmonary Medicine, Post code: 34360 Capa, Istanbul, Turkey.

Email: [email protected]

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Aylin Pihtili MD,

Aylin Pihtili MD

Istanbul Faculty of Medicine, Department of Pulmonary Medicine, Istanbul University, Istanbul, Turkey

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Zuleyha Bingol MD,

Zuleyha Bingol MD

Istanbul Faculty of Medicine, Department of Pulmonary Medicine, Istanbul University, Istanbul, Turkey

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Hacer Durmus MD,

Hacer Durmus MD

Istanbul Faculty of Medicine, Department of Neurology, Istanbul University, Istanbul, Turkey

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Yesim Parman MD,

Yesim Parman MD

Istanbul Faculty of Medicine, Department of Neurology, Istanbul University, Istanbul, Turkey

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Esen Kiyan MD,

Corresponding Author

Esen Kiyan MD

[email protected]

Istanbul Faculty of Medicine, Department of Pulmonary Medicine, Istanbul University, Istanbul, Turkey

Correspondence

Esen Kiyan, Istanbul University, Istanbul Faculty of Medicine, Department of Pulmonary Medicine, Post code: 34360 Capa, Istanbul, Turkey.

Email: [email protected]

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First published: 11 February 2021

https://doi.org/10.1002/mus.27200

Citations: 4

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Abstract

Introduction

In this study, we aimed to evaluate diaphragmatic dysfunction (DD) by using a practical approach in patients with amyotrophic lateral sclerosis (ALS) at the first visit to a chest diseases outpatient clinic.

Methods

Patients with ALS seen in our outpatient clinic for the past 5 y and followed up for at least 1 y, were retrospectively evaluated. Having at least one of the following three criteria was accepted as DD: (a) paradoxical abdominal movement (PAM), (b) sitting-supine forced vital capacity (FVC) difference ≥ 20%, (c) sitting-supine arterial oxygen saturation measured by pulse oximetry (SpO₂) difference ≥ 4%. Respiratory symptoms, arterial blood gas analysis, sleep studies, noninvasive mechanical ventilation use, and mortality were recorded.

Results

Five-hundred patients with ALS were included (female/male: 220/280, age: 58.9 ± 11.3 y). Of the patients, 22.8% had daytime hypercapnia. DD was observed in 55% of the patients (PAM in 112, sitting-supine FVC difference ≥ 20% in 50, and sitting-supine SpO₂ difference ≥ 4% in 113 patients). Of the patients with DD, 31.6% (n = 87) had no respiratory symptoms, 46.4% had FVC > 70% and 33.5% had FVC <50%. Nocturnal hypoxemia (sleep time spent with SpO₂ < 90% ≥30%) was present in 59.7%, and all patients with nocturnal hypoxemia had DD. Obstructive sleep apnea (8 severe, 14 moderate, 39 mild) was detected in 55% of the patients with polysomnography (n = 61) or polygraphy (n = 50). During follow-up, 52.2% of the patients died. Mean survival time was shorter in patients with DD (P < .001).

Conclusion

Paradoxical abdomimal movement (PAM), sitting-supine SpO₂ difference ≥ 4% and sitting-supine FVC difference ≥ 20% are indicators of DD, which should be routinely evaluated at every outpatient visit.

CONFLICT OF INTEREST

None of the authors has any conflict of interest to disclose.

Open Research

DATA AVAILABILITY STATEMENT

Author elects to not share data.

REFERENCES

1Spataro R, LoRe M, Piccoli T, Piccoli F, La Bella V. Causes and place of death in Italian patients with amyotrophic lateral sclerosis. Acta Neurol Scand. 2010; 122(3): 217-223.
10.1111/j.1600-0404.2009.01290.x
CAS PubMed Web of Science® Google Scholar
2Kurian KM, Forbes RB, Colville S, Swingler RJ. Cause of death and clinical grading criteria in a cohort of amyotrophic lateral sclerosis cases undergoing autopsy from the Scottish motor neurone disease register. J Neurol Neurosurg Psychiatry. 2009; 80(1): 84-87.
10.1136/jnnp.2008.149708
CAS PubMed Web of Science® Google Scholar
3Corcia P, Pradat PF, Salachas F, et al. Causes of death in a post-mortem series of ALS patients. Amyotroph Lateral Scler. 2008; 9(1): 59-62.
10.1080/17482960701656940
PubMed Google Scholar
4Louwerse ES, Visser CE, Bossuyt PM, Weverling GJ. Amyotrophic lateral sclerosis: mortality risk during the course of the disease and prognostic factors. J Neurol Sci. 1997; 152(1): 10-17.
10.1016/S0022-510X(97)00238-4
Web of Science® Google Scholar
5Arnulf 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(3): 849-856.
10.1164/ajrccm.161.3.9805008
CAS PubMed Web of Science® Google Scholar
6Bourke SC, Shaw PJ, Gibson GJ. Respiratory function vs. sleep-disordered breathing as predictors of QoL in ALS. Neurology. 2001; 57(11): 2040-2044.
10.1212/WNL.57.11.2040
CAS PubMed Web of Science® Google Scholar
7Mendoza M, Gelinas DF, Moore DH, Miller RG. A comparison of maximal inspiratory pressure and forced vital capacity as potential criteria for initiating non-invasive ventilation in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2007; 8(2): 106-111.
10.1080/17482960601030188
PubMed Web of Science® Google Scholar
8Czaplinski A, Yen AA, Appel SH. Forced vital capacity (FVC) as an indicator of survival and disease progression in an ALS clinic population. J Neurol Neurosurg Psychiatry. 2006; 77(3): 390-392.
10.1136/jnnp.2005.072660
CAS PubMed Web of Science® Google Scholar
9Miller RG, Jackson CE, Kasarskis EJ, et al. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence based review): report of the quality standards Subcommittee of the American Academy of neurology. Neurology. 2009; 73(15): 1218-1226.
10.1212/WNL.0b013e3181bc0141
CAS PubMed Web of Science® Google Scholar
10Lechtzin N, Wiener CM, Shade DM, Clawson L, Diette GB. Spirometry in the supine position improves the detection of diaphragmatic weakness in patients with amyotrophic lateral sclerosis. Chest. 2002; 121(2): 436-442.
10.1378/chest.121.2.436
PubMed Web of Science® Google Scholar
11Similowski T, Attali V, Bensimon G, et al. Diaphragmatic dysfunction and dyspnoea in amyotrophic lateral sclerosis. Eur Respir J. 2000; 15(2): 332-337.
10.1034/j.1399-3003.2000.15b19.x
CAS PubMed Web of Science® Google Scholar
12Andersen PM, Abrahams S, Borasio GD, et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)–revised report of an EFNS task force on diagnosis and Management of Amyotrophic Lateral Sclerosis. Eur J Neurol. 2012; 19(3): 360-375.
10.1111/j.1468-1331.2011.03501.x
PubMed Web of Science® Google Scholar
13Graham BL, Steenbruggen I, Miller MR, et al. Standardization of spirometry 2019 update. An official American Thoracic Society and European Respiratory Society technical statement. Am J Respir Crit Care Med. 2019; 200(8): 70-88.
10.1164/rccm.201908-1590ST
PubMed Web of Science® Google Scholar
14Lechtzin N, Cudkowicz ME, de Carvalho M, et al. Respiratory measures in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. 2018; 19(5–6): 321-330.
10.1080/21678421.2018.1452945
PubMed Web of Science® Google Scholar
15Kuenzel A, Marshall B, Verges S, Anholm JD. Positional changes in arterial oxygen saturation and end-tidal carbon dioxide at high altitude: medex 2015. High Alt Med Biol. 2020; 21(2): 144-151.
10.1089/ham.2019.0066
PubMed Web of Science® Google Scholar
16Ceylan B, Khorshid L, Güneş ÜY, Zaybak A. Evaluation of oxygen saturation values in different body positions in healthy individuals. J Clin Nurs. 2016; 25(7–8): 1095-1100.
10.1111/jocn.13189
PubMed Web of Science® Google Scholar
17O'Driscoll BR, Howard LS, Davison AG, British Thoracic Society. BTS guideline for emergency oxygen use in adult patients. Thorax. 2008; 63(Suppl 6): vi1-vi68.
PubMed Web of Science® Google Scholar
18Berry RB, Budhiraja R, Gottlieb DJ, et al. Rules for scoring respiratory events in sleep: update of the 2007 AASM manual for the scoring of sleep and associated events. Deliberations of the sleep Apnea definitions task force of the American Academy of sleep medicine. J Clin Sleep Med. 2012; 8(5): 597-619.
10.5664/jcsm.2172
PubMed Web of Science® Google Scholar
19Prell T, Ringer TM, Wullenkord K, et al. Assessment of pulmonary function in amyotrophic lateral sclerosis: when can polygraphy help evaluate the need for non-invasive ventilation? Neurol Neurosurg Psychiatry. 2016; 87(9): 1022-1026.
10.1136/jnnp-2015-312185
PubMed Web of Science® Google Scholar
20Schiffman PL, Belsh JM. Pulmonary function at diagnosis of amyotrophic lateral sclerosis. Rate of deterioration. Chest. 1993; 103(2): 508-513.
10.1378/chest.103.2.508
CAS PubMed Web of Science® Google Scholar
21De Carvalho M, Matias T, Coelho F, Evangelista T, Pinto A, Luís MS. Motor neuron disease presenting with respiratory failure. J Neurol Sci. 1996; 139(1): 117-122.
10.1016/0022-510X(96)00089-5
PubMed Web of Science® Google Scholar
22Dubé BP, Dres M. Diaphragm dysfunction: diagnostic approaches and management strategies. J Clin Med. 2016; 5(12): 113.
10.3390/jcm5120113
Web of Science® Google Scholar
23Harlaar L, Ciet P, van der Ploeg AT, et al. Imaging of respiratory muscles in neuromuscular disease: a review. Neuromuscul Disord. 2018; 28(3): 246-256.
10.1016/j.nmd.2017.11.010
CAS PubMed Web of Science® Google Scholar
24De Carvalho M, Swash M, Pinto S. Diaphragmatic neurophysiology and respiratory markers in ALS. Front Neurol. 2019; 10: 143.
10.3389/fneur.2019.00143
PubMed Web of Science® Google Scholar
25Baumann F, Henderson RD, Morrison SC, et al. Use of respiratory function tests to predict survival in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2010; 11(1–2): 194-202.
10.3109/17482960902991773
PubMed Google Scholar
26Schmidt EP, Drachman DB, Wiener CM, Clawson L, Kimball R, Lechtzin N. Pulmonary predictors of survival in amyotrophic lateral sclerosis: use in clinical trial design. Muscle Nerve. 2006; 33(1): 127-132.
10.1002/mus.20450
PubMed Web of Science® Google Scholar
27Pinto S, Turkman A, Pinto A, Swash M, de Carvalho M. Predicting respiratory insufficiency in amyotrophic lateral sclerosis: the role of phrenic nevre studies. Clin Neurophysiol. 2009; 120(5): 941-946.
10.1016/j.clinph.2009.02.170
PubMed Web of Science® Google Scholar
28Fantini R, Mandrioli J, Zona S, et al. Ultrasound assessment of diaphragmatic function in patients with amyotrophic lateral sclerosis. Respirology. 2016; 21(5): 932-938.
10.1111/resp.12759
PubMed Web of Science® Google Scholar
29Sartucci F, Pelagatti A, Santin M, Bocci T, Dolciotti C, Bongioanni P. Diaphragm ultrasonography in amyotrophic lateral sclerosis: a diagnostic tool to assess ventilatory dysfunction and disease severity. Neurol Sci. 2019; 40(10): 2065-2071.
10.1007/s10072-019-03938-9
PubMed Web of Science® Google Scholar
30Pinto S, Alves P, Pimentel B, Swash M, de Carvalho M. Ultrasound for assessment of diaphragm in ALS. Clin Neurophysiol. 2016; 127(1): 892-897.
10.1016/j.clinph.2015.03.024
PubMed Web of Science® Google Scholar
31Pinto S, Carvalho M. Correlation between forced vital capacity and slow vital capacity for the assessment of respiratory involvement in amyotrophic lateral sclerosis: a prospective study. Amyotroph Lateral Scler Frontotemporal Degener. 2017; 18(1–2): 86-91.
10.1080/21678421.2016.1249486
PubMed Web of Science® Google Scholar
32Rose L, McKim D, Leasa D, et al. Patterns of healthcare utilisation for respiratory complications of adults with neuromuscular disease: a population study. Eur Respir J. 2018; 52(3):1800754.
10.1183/13993003.00754-2018
PubMed Web of Science® Google Scholar
33D'Cruz RF, Murphy PB, Kaltsakas G. Sleep disordered breathing in motor neurone disease. J Thorac Dis. 2018; 10(1): 86-93.
10.21037/jtd.2017.12.19
Web of Science® Google Scholar
34Braun AT, Caballero-Eraso C, Lechtzin N. Amyotrophic lateral sclerosis and the respiratory system. Clin Chest Med. 2018; 39(2): 391-400.
10.1016/j.ccm.2018.01.003
PubMed Web of Science® Google Scholar
35Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol. 2006; 5(2): 140-147.
10.1016/S1474-4422(05)70326-4
PubMed Web of Science® Google Scholar
36Iłzecka J, Stelmasiak Z, Balicka G. Respiratory function in amyotrophic lateral sclerosis. Neurol Sci. 2003; 24(4): 288-289.
10.1007/s10072-003-0159-2
CAS PubMed Web of Science® Google Scholar

Citing Literature

Volume63, Issue5

May 2021

Pages 683-689

Diaphragmatic dysfunction at the first visit to a chest diseases outpatient clinic in 500 patients with amyotrophic lateral sclerosis

Abstract

Introduction

Methods

Results

Conclusion

CONFLICT OF INTEREST

Open Research

DATA AVAILABILITY STATEMENT

REFERENCES

Citing Literature

References

Information

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Diaphragmatic dysfunction at the first visit to a chest diseases outpatient clinic in 500 patients with amyotrophic lateral sclerosis

Abstract

Introduction

Methods

Results

Conclusion

CONFLICT OF INTEREST

Open Research

DATA AVAILABILITY STATEMENT

REFERENCES

Citing Literature

References

Related

Information