nach oben

Erschienen in:

12.02.2020 | Original Communication

Prognostic role of slow vital capacity in amyotrophic lateral sclerosis

verfasst von: Andrea Calvo, Rosario Vasta, Cristina Moglia, Enrico Matteoni, Antonio Canosa, Alessio Mattei, Claudio La Mancusa, Luana Focaraccio, Letizia Mazzini, Adriano Chiò, Fabrizio D’Ovidio, Umberto Manera

Erschienen in: Journal of Neurology | Ausgabe 6/2020

Einloggen, um Zugang zu erhalten

Abstract

Objectives

To compare the prognostic role of FVC and SVC at diagnosis in amyotrophic lateral sclerosis (ALS) patients.

Methods

We included all patients from the Piemonte and Valle D’Aosta ALS register (PARALS) who had been diagnosed with ALS between 1995 and 2015 and underwent spirometry at diagnosis. Survival was considered as time to death/tracheostomy; to assess the prognostic value in typical trial timeframes, survival at 12 and 18 months was calculated too. Cox proportional hazard regression models adjusted by sex, age at diagnosis, diagnostic delay, onset site, and ALSFRS-R total score at the moment of diagnosis were used to assess the prognostic role of FVC and SVC.

Results

A total of 795 ALS patients underwent spirometry at diagnosis during the study period. Four hundred and sixteen (52.3%) performed both FVC and SVC, whereas the others performed FVC only. FVC and SVC values were highly correlated (r = 0.92, p < 0.001) in the overall population and slightly less correlated in patients with bulbar onset (r = 0.86, p < 0.001). Both FVC and SVC proved to have a prognostic role with comparable hazard ratios (HRs) (HR 1.83, 95% CI 1.48–2.27 and 1.88, 95% CI 1.51–2.33, respectively). When considering typical trial timeframes, HRs remained similar and were inversely proportional to FVC and SVC values.

Discussion

FVC and SVC at diagnosis can be used interchangeably as independent predictors of survival in both clinical and research settings.

Nur mit Berechtigung zugänglich

van Es MA, Hardiman O, Chio A et al (2017) Amyotrophic lateral sclerosis. Lancet 390:2084–2098. https://doi.org/10.1016/S0140-6736(17)31287-4 CrossRefPubMed

Amyotrophic Lateral Sclerosis (ALS) Fact Sheet|National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet. Accessed 20 Mar 2019

Pinto S, de Carvalho M (2015) The R of ALSFRS-R: does it really mirror functional respiratory involvement in amyotrophic lateral sclerosis? Amyotroph Lateral Scler Front Degener 16:120–123. https://doi.org/10.3109/21678421.2014.952641 CrossRef

Lenglet T, Lacomblez L, Abitbol JL et al (2014) A phase II–III trial of olesoxime in subjects with amyotrophic lateral sclerosis. Eur J Neurol 21:529–536. https://doi.org/10.1111/ene.12344 CrossRefPubMed

Czaplinski A, Yen AA, Appel SH (2006) Forced vital capacity (FVC) as an indicator of survival and disease progression in an ALS clinic population. J Neurol Neurosurg Psychiatry 77:390–392. https://doi.org/10.1136/jnnp.2005.072660 CrossRefPubMedPubMedCentral

Tilanus TBM, Groothuis JT, TenBroek-Pastoor JMC et al (2017) The predictive value of respiratory function tests for non-invasive ventilation in amyotrophic lateral sclerosis. Respir Res 18:144. https://doi.org/10.1186/s12931-017-0624-8 CrossRefPubMedPubMedCentral

Pinto S, De Carvalho M (2015) Sessions 1–11. Amyotroph Lateral Scler Front Degener 16:1–62. https://doi.org/10.3109/21678421.2015.1089039 CrossRef

Magnus T, Beck M, Giess R et al (2002) Disease progression in amyotrophic lateral sclerosis: predictors of survival. Muscle Nerve 25:709–714. https://doi.org/10.1002/mus.10090 CrossRefPubMed

Andrews JA, Meng L, Kulke SF et al (2018) Association between decline in slow vital capacity and respiratory insufficiency, use of assisted ventilation, tracheostomy, or death in patients with amyotrophic lateral sclerosis. JAMA Neurol 75:58–64. https://doi.org/10.1001/jamaneurol.2017.3339 CrossRefPubMed

10.

Singh D, Verma R, Garg RK et al (2011) Assessment of respiratory functions by spirometry and phrenic nerve studies in patients of amyotrophic lateral sclerosis. J Neurol Sci 306:76–81. https://doi.org/10.1016/j.jns.2011.03.039 CrossRefPubMed

11.

Lyall RA, Donaldson N, Polkey MI et al (2001) Respiratory muscle strength and ventilatory failure in amyotrophic lateral sclerosis. Brain J Neurol 124:2000–2013CrossRef

12.

Jackson C, De Carvalho M, Genge A et al (2018) Relationships between slow vital capacity and measures of respiratory function on the ALSFRS-R. Amyotroph Lateral Scler Front Degener. https://doi.org/10.1080/21678421.2018.1497658 CrossRef

13.

Enache I, Pistea C, Fleury M et al (2017) Ability of pulmonary function decline to predict death in amyotrophic lateral sclerosis patients. Amyotroph Lateral Scler Front Degener 18:511–518. https://doi.org/10.1080/21678421.2017.1353097 CrossRef

14.

Baumann F, Henderson RD, Morrison SC et al (2010) Use of respiratory function tests to predict survival in amyotrophic lateral sclerosis. Amyotroph Lateral Scler 11:194–202. https://doi.org/10.3109/17482960902991773 CrossRefPubMed

15.

Pinto S, de Carvalho M (2017) 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 Front Degener 18:86–91. https://doi.org/10.1080/21678421.2016.1249486 CrossRef

16.

Traynor BJ, Zhang H, Shefner JM et al (2004) Functional outcome measures as clinical trial endpoints in ALS. Neurology 63:1933–1935CrossRefPubMed

17.

Kaufmann P, Levy G, Thompson JLP et al (2005) The ALSFRSr predicts survival time in an ALS clinic population. Neurology 64:38–43. https://doi.org/10.1212/01.WNL.0000148648.38313.64 CrossRefPubMed

18.

Pinto S, de Carvalho M (2019) SVC is a marker of respiratory decline function, similar to FVC, in patients with ALS. Front Neurol 10:109. https://doi.org/10.3389/fneur.2019.00109 CrossRefPubMedPubMedCentral

19.

Pinto S, de Carvalho M (2017) Comparison of slow and forced vital capacities on ability to predict survival in ALS. Amyotroph Lateral Scler Front Degener 18:528–533. https://doi.org/10.1080/21678421.2017.1354995 CrossRef

20.

Chiò A, Mora G, Moglia C et al (2017) Secular trends of amyotrophic lateral sclerosis: the Piemonte and Valle d’Aosta Register. JAMA Neurol. https://doi.org/10.1001/jamaneurol.2017.1387 CrossRefPubMedPubMedCentral

21.

Cedarbaum JM, Stambler N, Malta E et al (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 169:13–21CrossRefPubMed

22.

Manera U, Calvo A, Daviddi M et al (2019) Regional spreading of symptoms at diagnosis as a prognostic marker in amyotrophic lateral sclerosis: a population-based study. J Neurol Neurosurg Psychiatry. https://doi.org/10.1136/jnnp-2019-321153 CrossRefPubMed

23.

EFNS Task Force on Diagnosis, and Management of Amyotrophic Lateral Sclerosis, Andersen PM, Abrahams S et al (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. https://doi.org/10.1111/j.1468-1331.2011.03501.x CrossRef

24.

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

25.

Lechtzin N, Wiener CM, Shade DM et al (2002) Spirometry in the supine position improves the detection of diaphragmatic weakness in patients with amyotrophic lateral sclerosis. Chest 121:436–442CrossRefPubMed

26.

Caruso P, de Albuquerque ALP, Santana PV et al (2015) Diagnostic methods to assess inspiratory and expiratory muscle strength. J Bras Pneumol 41:110–123. https://doi.org/10.1590/S1806-37132015000004474 CrossRefPubMedPubMedCentral

27.

Jackson CE, Rosenfeld J, Moore DH et al (2001) A preliminary evaluation of a prospective study of pulmonary function studies and symptoms of hypoventilation in ALS/MND patients. J Neurol Sci 191:75–78CrossRefPubMed

28.

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

29.

Pinto S, de Carvalho M (2018) Sniff nasal inspiratory pressure (SNIP) in amyotrophic lateral sclerosis: relevance of the methodology for respiratory function evaluation. Clin Neurol Neurosurg 171:42–45. https://doi.org/10.1016/j.clineuro.2018.05.011 CrossRefPubMed

30.

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

31.

Morgan RK, McNally S, Alexander M et al (2005) Use of Sniff nasal-inspiratory force to predict survival in amyotrophic lateral sclerosis. Am J Respir Crit Care Med 171:269–274. https://doi.org/10.1164/rccm.200403-314OC CrossRefPubMed

32.

Matsuda C, Shimizu T, Nakayama Y, Haraguchi M (2019) Cough peak flow decline rate predicts survival in patients with amyotrophic lateral sclerosis. Muscle Nerve 59:168–173. https://doi.org/10.1002/mus.26320 CrossRefPubMed

33.

Pinto S, Pinto A, de Carvalho M (2012) Phrenic nerve studies predict survival in amyotrophic lateral sclerosis. Clin Neurophysiol 123:2454–2459. https://doi.org/10.1016/j.clinph.2012.05.012 CrossRefPubMed

34.

Boentert M, Glatz C, Helmle C et al (2018) Prevalence of sleep apnoea and capnographic detection of nocturnal hypoventilation in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 89:418–424. https://doi.org/10.1136/jnnp-2017-316515 CrossRefPubMed

35.

Rafiq MK, Bradburn M, Proctor AR et al (2012) Using transcutaneous carbon dioxide monitor (TOSCA 500) to detect respiratory failure in patients with amyotrophic lateral sclerosis: a validation study. Amyotroph Lateral Scler 13:528–532. https://doi.org/10.3109/17482968.2012.688836 CrossRefPubMed

36.

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

37.

Chiò A, Canosa A, Gallo S et al (2011) ALS clinical trials: do enrolled patients accurately represent the ALS population? Neurology 77:1432–1437. https://doi.org/10.1212/WNL.0b013e318232ab9b CrossRefPubMed

38.

Van Eijk RPA, Westeneng H-J, Nikolakopoulos S et al (2019) Refining eligibility criteria for amyotrophic lateral sclerosis clinical trials. Neurology. https://doi.org/10.1212/WNL.0000000000006855 CrossRefPubMedPubMedCentral

Titel: Prognostic role of slow vital capacity in amyotrophic lateral sclerosis
verfasst von: Andrea Calvo
Rosario Vasta
Cristina Moglia
Enrico Matteoni
Antonio Canosa
Alessio Mattei
Claudio La Mancusa
Luana Focaraccio
Letizia Mazzini
Adriano Chiò
Fabrizio D’Ovidio
Umberto Manera
Publikationsdatum: 12.02.2020
Verlag: Springer Berlin Heidelberg
Erschienen in: Journal of Neurology / Ausgabe 6/2020
Print ISSN: 0340-5354
Elektronische ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-020-09751-1

Leitlinien kompakt für die Neurologie

Mit medbee Pocketcards sicher entscheiden.

^{Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag}

Kostenlos registrieren

Neu im Fachgebiet Neurologie

Wartezeit nicht kürzer, aber Arbeit flexibler

Psychotherapie Medizin aktuell

Fünf Jahren nach der Neugestaltung der Psychotherapie-Richtlinie wurden jetzt die Effekte der vorgenommenen Änderungen ausgewertet. Das Hauptziel der Novellierung war eine kürzere Wartezeit auf Therapieplätze. Dieses Ziel wurde nicht erreicht, es gab jedoch positive Auswirkungen auf andere Bereiche.

Update Neurologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Springer Medizin

Prognostic role of slow vital capacity in amyotrophic lateral sclerosis