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Sniff nasal inspiratory pressure as a prognostic factor of tracheostomy or death in amyotrophic lateral sclerosis

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Abstract

Forced vital capacity (FVC) shows limitations in detecting respiratory failure in the early phase of amyotrophic lateral sclerosis (ALS). In fact, mild-to-moderate respiratory muscle weakness may be present even when FVC is normal, and ALS patients with bulbar involvement might not be able to perform correctly the spirometry test. Sniff nasal inspiratory pressure (SNIP) is correlated with transdiaphragmatic strength. We evaluated SNIP at baseline as a prognostic factor of tracheostomy or death in patients with ALS. In a multidisciplinary tertiary care center for motorneuron disease, we enrolled 100 patients with ALS diagnosed with El Escorial criteria in the period between January 2006 and December 2010. Main outcome measures were tracheostomy or death. RECursive Partitioning and AMalgamation (RECPAM) analysis was also used to identify subgroups at different risks for the tracheostomy or death. Twenty-nine patients with ALS reached the outcome (12 died and 17 had tracheostomy). Using a multivariate model SNIP correctly classified the risk of the composite event within 1 year of follow-up with a continuous Net Reclassification Improvement cNRI of 0.58 (p = 0.03). Sex, Amyotrophic Lateral Sclerosis Functional Rating Scale revisited, site of onset, and FVC did not improve the classification of prognostic classes. SNIP ≤18 cmH2O identified the RECPAM class with the highest risk (Class 1, hazard ratio = 9.85, 95 % confidence interval: 2.67–36.29, p < 0.001). SNIP measured at baseline identified patients with ALS with initial respiratory failure. Finally, using only ALS patients with spinal onset of the disease, our findings were mostly overlapping with those reported in the models including the whole sample. At baseline, SNIP appeared to be the best predictor of death or tracheostomy within 1 year of follow-up. The measurement of SNIP in the early phase of the disease may contribute to identify patients with high risk of mortality or intubation. SNIP may also provide an additional tool for baseline stratification of patients with ALS in clinical trials.

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Abbreviations

ALS:

Amyotrophic lateral sclerosis

ALSFRS-R:

Revised amyotrophic lateral sclerosis Functional Rating Scale

AUC:

Area under the receiver operating characteristic Curve

BDI:

Beck Depression Inventory

EI:

Executive index

FAB:

Frontal Assessment Battery

FVC:

Forced vital capacity

FTD:

Frontotemporal dementia

NIV:

Noninvasive ventilation

MMSE:

Mini-Mental State Examination

MMT:

Manual muscle testing

MoCA:

Montreal Cognitive Assessment

95 % CI:

95 % confidence intervals

RECPAM:

RECursive Partitioning and Amalgamation

ROC:

Receiver-operating characteristic

SD:

Standard deviation

SDMT:

Symbol Digit Modalities Test-Oral version

ST:

Stroop test

VFT:

Verbal Fluency Test

References

  1. Traynor BJ, Zhang H, Shefner JM, Schoenfeld D, Cudkowicz ME, NEALS Consortium (2004) Functional outcome measures as clinical trial endpoints in ALS. Neurology 63:1933–1935

    Article  CAS  PubMed  Google Scholar 

  2. Hardiman O, Kollewe K, Leigh PN, Pradat PF, Silani V, Tomik B, EALSC Working Group (2007) Good practice in the management of amyotrophic lateral sclerosis: clinical guidelines. An evidence-based review with good practice points. EALSC Working Group. Amyotroph Lateral Scler 8:195–213

    Article  PubMed  Google Scholar 

  3. Kiernan MC, Vucic S, Cheah BC, Turner MR, Eisen A, Hardiman O, Burrell JR, Zoing MC (2011) Amyotrophic lateral sclerosis. Lancet 377:942–955

    Article  CAS  PubMed  Google Scholar 

  4. Chiò A, Logroscino G, Hardiman O, Swingler R, Mitchell D, Beghi E, Traynor BG, Eurals Consortium (2009) Prognostic factors in ALS: a critical review. Amyotroph Lateral Scler 10:310–323

    Article  PubMed Central  PubMed  Google Scholar 

  5. Schiffman PL, Belsh JM (1993) Pulmonary function at diagnosis of amyotrophic lateral sclerosis. Chest 103:508–513

    Article  CAS  PubMed  Google Scholar 

  6. Fallat RJ, Jewitt B, Bass M, Kamm B, Norris FH Jr (1979) Spirometry in amyotrophic lateral sclerosis. Arch Neurol 36:74–78

    Article  CAS  PubMed  Google Scholar 

  7. Gordon PH, Moore DH, Miller RG, Florence JM, Verheijde JL, Doorish C, Hilton JF, Spitalny GM, MacArthur RB, Mitsumoto H, Neville HE, Boylan K, Mozaffar T, Belsh JM, Ravits J, Bedlack RS, Graves MC, McCluskey LF, Barohn RJ, Tandan R, Western ALS Study Group (2007) Efficacy of minocycline in patients with amyotrophic lateral sclerosis: a phase III randomised trial. Lancet Neurol 6:1045–1053

    Article  CAS  PubMed  Google Scholar 

  8. UKMND-LiCALS Study Group, Morrison KE, Dhariwal S, Hornabrook R, Savage L, Burn DJ, Khoo TK, Kelly J, Murphy CL, Al-Chalabi A, Dougherty A, Leigh PN, Wijesekera L, Thornhill M, Ellis CM, O’Hanlon K, Panicker J, Pate L, Ray P, Wyatt L, Young CA, Copeland L, Ealing J, Hamdalla H, Leroi I, Murphy C, O’Keeffe F, Oughton E, Partington L, Paterson P, Rog D, Sathish A, Sexton D, Smith J, Vanek H, Dodds S, Williams TL, Steen IN, Clarke J, Eziefula C, Howard R, Orrell R, Sidle K, Sylvester R, Barrett W, Merritt C, Talbot K, Turner MR, Whatley C, Williams C, Williams J, Cosby C, Hanemann CO, Iman I, Philips C, Timings L, Crawford SE, Hewamadduma C, Hibberd R, Hollinger H, McDermott C, Mils G, Rafiq M, Shaw PJ, Taylor A, Waines E, Walsh T, Addison-Jones R, Birt J, Hare M, Majid T (2013) Lithium in patients with amyotrophic lateral sclerosis (LiCALS): a phase 3 multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol 12:339–345

    Article  PubMed Central  Google Scholar 

  9. Fitting JW, Paillex R, Hirt L, Aebischer P, Schluep M (1999) Sniff nasal pressure: a sensitive respiratory test to assess progression of amyotrophic lateral sclerosis. Ann Neurol 46:887–893

    Article  CAS  PubMed  Google Scholar 

  10. Black LF, Hyatt RE (1971) Maximal static respiratory pressures in generalized neuromuscular disease. Am Rev Respir Dis 103:641–650

    CAS  PubMed  Google Scholar 

  11. Polkey MI, Green M, Moxham J (1995) Measurement of respiratory muscle strength. Thorax 50:1131–1135

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  12. Miller JM, Moxham J, Green M (1985) The maximal sniff in the assessment of diaphragm function in man. Clin Sci (Lond) 69:91–96

    Article  CAS  Google Scholar 

  13. Lyall RA, Donaldson N, Polkey MI, Leigh PN, Moxham J (2001) Respiratory muscle strength and ventilatory failure in amyotrophic lateral sclerosis. Brain 124:2000–2013

    Article  CAS  PubMed  Google Scholar 

  14. Stefanutti D, Benoist MR, Scheinmann P, Chaussain M, Fitting JW (2000) Usefulness of sniff nasal pressure in patients with neuromuscular or skeletal disorders. Am J Respir Crit Care Med 162:1507–1511

    Article  CAS  PubMed  Google Scholar 

  15. Schmidt EP, Drachman DB, Wiener CM, Clawson L, Kimball R, Lechtzin N (2006) Pulmonary predictors of survival in amyotrophic lateral sclerosis: use in clinical trial design. Muscle Nerve 33:127–132

    Article  PubMed  Google Scholar 

  16. Morgan RK, McNally S, Alexander M, Conroy R, Hardiman O, Costello RW (2005) Use of Sniff nasal-inspiratory force to predict survival in amyotrophic lateral sclerosis. Am J Respir Crit Care Med 171:269–274

    Article  PubMed  Google Scholar 

  17. Von Elm E, Altman DG, Egger M, Pocock SJ, Gøtzsche PC, Vandenbroucke JP, STROBE Initiative (2007) Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) statement: guidelines for reporting observational studies. BMJ 335:806–808

    Article  Google Scholar 

  18. Brooks BR (1994) El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on Motor Neuron Diseases/Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases and the El Escorial “Clinical limits of amyotrophic lateral sclerosis” workshop contributors. J Neurol Sci 124(Suppl):96–107

    Article  PubMed  Google Scholar 

  19. Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, Nakanishi A (1999) The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci 169:13–21

    Article  CAS  PubMed  Google Scholar 

  20. Charlson ME, Pompei P, Ales KL, MacKenzie CR (1987) A new method of classifying prognostic comorbidity in longitudinal studies: development and validation. J Chronic Dis 40:373–383

    Article  CAS  PubMed  Google Scholar 

  21. Miller MR, Hankinson J, Brusasco V, Crapo RO, Burgos F, Casaburi R, Coates A, van der Grinten CP, Gustafsson P, Hankinson J, Jensen R, Johnson DC, MacIntyre N, McKay R, Miller MR, Navajas D, Pedersen OF, Wanger J, ATS, ERS Task Force (2005) Standardisation of spirometry. Eur Respir J 26:319–338

    Article  CAS  PubMed  Google Scholar 

  22. American Thoracic Society/European Respiratory Society (2002) ATS/ERS Statement on respiratory muscle testing. Am J Respir Crit Care Med 166:518–624

    Article  Google Scholar 

  23. Pencina MJ, D’Agostino RB, Steyerberg EW (2011) Extensions of net reclassification improvement calculations to measure usefulness of new biomarkers. Stat Med 30:11–21

    Article  PubMed Central  PubMed  Google Scholar 

  24. del Aguila MA, Longstreth WT Jr, McGuire V, Koepsell TD, van Belle G (2003) Prognosis in amyotrophic lateral sclerosis. A population-based study. Neurology 60:813–819

    Article  PubMed  Google Scholar 

  25. Wolf J, Safer A, Wöhrle JC, Palm F, Nix WA, Maschke M, Grau AJ (2014) Factors predicting one-year mortality in amyotrophic lateral sclerosis patients—data from a population-based registry. BMC Neurol 14:197

    Article  PubMed Central  PubMed  Google Scholar 

  26. Polkey MI, Lyall RA, Green M, Nigel Leigh P, Moxham J (1998) Expiratory muscle function in amyotrophic lateral sclerosis. Am J Respir Crit Care Med 158:734–741

    Article  CAS  PubMed  Google Scholar 

  27. Kleopa KA, Sherman M, Neal B, Romano GJ, Heiman-Patterson T (1999) Bipap improves survival and rate of pulmonary function decline in patients with ALS. J Neurol Sci 164:82–88

    Article  CAS  PubMed  Google Scholar 

  28. Fregonezi G, Araújo PR, Macêdo TL, Dourado Junior ME, Resqueti VR, Andrade Ade F (2013) Monitoring respiratory muscle strength assists in early diagnosis of respiratory dysfunction as opposed to the isolated use of pulmonary function evaluation in amyotrophic lateral sclerosis. Arq Neuropsiquiatr 71:146–152

    Article  PubMed  Google Scholar 

  29. Baumann F, Henderson RD, Morrison SC, Brown M, Hutchinson N, Douglas JA, Robinson PJ, McCombe PA (2010) Use of respiratory function tests to predict survival in amyotrophic lateral sclerosis. Amyotroph Lateral Scler 11:194–202

    Article  PubMed  Google Scholar 

  30. Ward NS, Hill NS (2001) Pulmonary function testing in neuromuscular disease. Clin Chest Med 22:769–781

    Article  CAS  PubMed  Google Scholar 

  31. Vincken W, Elleker G, Cosio MG (1986) Detection of upper airway muscle involvement in neuromuscular disorders using the flow-volume loop. Chest 90:52–57

    Article  CAS  PubMed  Google Scholar 

  32. Carratù P, Cassano A, Gadaleta F et al (2011) Association between low sniff nasal-inspiratory pressure (SNIP) and sleep disordered breathing in amyotrophic lateral sclerosis: preliminary results. Amyotroph Lateral Scler 12:458–463

    Article  PubMed  Google Scholar 

  33. Uldry C, Fitting JW (1995) Maximal values of sniff nasal inspiratory pressure in healthy subjects. Thorax 50:371–375

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  34. Logroscino G, Traynor BJ, Hardiman O, Chio’ A, Couratier P, Mitchell JD, Swingler RJ, Beghi E, EURALS, (2008) Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues. J Neurol Neurosurg Psychiatry 79:6–11

    Article  CAS  PubMed  Google Scholar 

  35. Paesmans M, Sculier JP, Libert P, Bureau G, Dabouis G, Thiriaux J, Michel J, Van Cutsem O, Sergysels R, Mommen P (1995) Prognostic factors for survival in advanced non-small-cell lung cancer: univariate and multivariate analyses including recursive partitioning and amalgamation algorithms in 1,052 patients. The European Lung Cancer Working Party. J Clin Oncol 13:1221–1230

    CAS  PubMed  Google Scholar 

  36. EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis, Andersen PM, Abrahams S, Borasio GD, de Carvalho M, Chio A, Van Damme P, Hardiman O, Kollewe K, Morrison KE, Petri S, Pradat PF, Silani V, Tomik B, Wasner M, Weber M (2012) EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)—revised report of an EFNS task force. Eur J Neurol 19:360–375

    Article  Google Scholar 

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Acknowledgments

This research was supported by European Community’s Seventh Framework Programme (FP7/2007-2013 under grant agreement 259867).

Conflicts of interest

The authors declare no financial or other conflicts of interest.

Ethical standard

The study has been approved by the Local Ethical Committee and has therefore been performed in accordance with the ethical standards laid down World Medical Association’s 2008 Declaration of Helsinki. All persons gave their informed consent prior to their inclusion in the study.

Author information

Authors and Affiliations

  1. Neuroscience and Sense Organs, Department of Basic Medical Science, Neurodegenerative Diseases Unit, University of Bari “Aldo Moro”, Bari, Italy

    Rosa Capozzo, Francesco Panza, Rosanna Tortelli, Rosa Cortese, Isabella L. Simone & Giancarlo Logroscino

  2. Pulmonary Disease Institute, University of Bari “Aldo Moro”, Bari, Italy

    Vitaliano N. Quaranta, Pierluigi Carratù, Anna Cassano, Vito A. Falcone & Onofrio Resta

  3. Unit of Biostatistics, IRCCS Casa Sollievo della Sofferenza, San Giovanni Rotondo, FG, Italy

    Fabio Pellegrini, Andrea Fontana & Massimiliano Copetti

  4. Unit of Biostatistics, Consorzio Mario Negri Sud, Santa Maria Imbaro, CH, Italy

    Fabio Pellegrini

  5. Department of Clinical Research in Neurology, University of Bari “Aldo Moro”, “Pia Fondazione Cardinale G. Panico”, Tricase, LE, Italy

    Francesco Panza, Rosanna Tortelli & Giancarlo Logroscino

Authors
  1. Rosa Capozzo
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  2. Vitaliano N. Quaranta
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  8. Anna Cassano
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  9. Vito A. Falcone
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  14. Giancarlo Logroscino
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Correspondence to Giancarlo Logroscino.

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Capozzo, R., Quaranta, V.N., Pellegrini, F. et al. Sniff nasal inspiratory pressure as a prognostic factor of tracheostomy or death in amyotrophic lateral sclerosis. J Neurol 262, 593–603 (2015). https://doi.org/10.1007/s00415-014-7613-3

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  • DOI: https://doi.org/10.1007/s00415-014-7613-3

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