Use of the combination of spirometry, arterial blood gas analysis and overnight oximetry to predict the outcomes of patients affected by motor neuron disease: The Milan-Torin respiratory score (Mi-To-RS).
ALS
NIMV
amyotrophic lateral sclerosis
blood gas analysis
neuromuscular diseases
non‐invasive ventilation
prognosis
respiratory failure
spirometry
Journal
European journal of neurology
ISSN: 1468-1331
Titre abrégé: Eur J Neurol
Pays: England
ID NLM: 9506311
Informations de publication
Date de publication:
08 May 2024
08 May 2024
Historique:
revised:
23
02
2024
received:
07
08
2023
accepted:
10
04
2024
medline:
8
5
2024
pubmed:
8
5
2024
entrez:
8
5
2024
Statut:
aheadofprint
Résumé
The use of multiple tests, including spirometry, arterial blood gas (ABG) analysis and overnight oximetry (OvOx), is highly recommended to monitor the respiratory function of patients with motor neuron disease (MND). In this study, we propose a composite score to simplify the respiratory management of MND patients and better stratify their prognosis. We screened the clinical charts of 471 non-ventilated MND patients referred to the Neuro-rehabilitation Unit of the San Raffaele Scientific Institute of Milan (January 2001-December 2019), collecting spirometric, ABG and OvOx parameters. To evaluate the prognostic role of each measurement, univariate Cox regression for death/tracheostomy was performed, and the variables associated with survival were selected to design a scoring system. Univariate and multivariate Cox regression analyses were then carried out to evaluate the prognostic role of the score. Finally, results were replicated in an independent cohort from the Turin ALS Center. The study population included 450 patients. Six measurements were found to be significantly associated with survival and were selected to design a scoring system (maximum score = 8 points). Kaplan-Meier analysis showed significant stratification of survival and time to non-invasive mechanical ventilation adaptation according to score values, and multivariate analysis confirmed the independent effect of the respiratory score on survival of each cohort. Forced vital capacity, ABG and OvOx parameters provide complementary information for the respiratory management and prognosis of MND patients and the combination of these parameters into a single score might help neurologists predict prognosis and guide decisions on the timing of the implementation of different diagnostic or therapeutic approaches.
Sections du résumé
BACKGROUND AND PURPOSE
OBJECTIVE
The use of multiple tests, including spirometry, arterial blood gas (ABG) analysis and overnight oximetry (OvOx), is highly recommended to monitor the respiratory function of patients with motor neuron disease (MND). In this study, we propose a composite score to simplify the respiratory management of MND patients and better stratify their prognosis.
MATERIALS AND METHODS
METHODS
We screened the clinical charts of 471 non-ventilated MND patients referred to the Neuro-rehabilitation Unit of the San Raffaele Scientific Institute of Milan (January 2001-December 2019), collecting spirometric, ABG and OvOx parameters. To evaluate the prognostic role of each measurement, univariate Cox regression for death/tracheostomy was performed, and the variables associated with survival were selected to design a scoring system. Univariate and multivariate Cox regression analyses were then carried out to evaluate the prognostic role of the score. Finally, results were replicated in an independent cohort from the Turin ALS Center.
RESULTS
RESULTS
The study population included 450 patients. Six measurements were found to be significantly associated with survival and were selected to design a scoring system (maximum score = 8 points). Kaplan-Meier analysis showed significant stratification of survival and time to non-invasive mechanical ventilation adaptation according to score values, and multivariate analysis confirmed the independent effect of the respiratory score on survival of each cohort.
CONCLUSION
CONCLUSIONS
Forced vital capacity, ABG and OvOx parameters provide complementary information for the respiratory management and prognosis of MND patients and the combination of these parameters into a single score might help neurologists predict prognosis and guide decisions on the timing of the implementation of different diagnostic or therapeutic approaches.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e16316Subventions
Organisme : Giovanni Marazzina Foundation
Informations de copyright
© 2024 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.
Références
Brown RH, Al‐Chalabi A. Amyotrophic lateral sclerosis. N Engl J Med. 2017;377:162‐172. doi:10.1056/NEJMra1603471
Riva N, Agosta F, Lunetta C, Filippi M, Quattrini A. Recent advances in amyotrophic lateral sclerosis. J Neurol. 2016;263(6):1241‐1254.
Miller 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.
Westeneng H‐J, Debray TPA, Visser AE, et al. Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model. Lancet Neurol. 2018;17(5):423‐433.
Niedermeyer S, Murn M, Choi PJ. Respiratory failure in amyotrophic lateral sclerosis. Chest. 2019;155(2):401‐408.
Kelly CR, Parra‐Cantu C, Thapa P, et al. Comparative performance of different respiratory test parameters for detection of early respiratory insufficiency in patients with ALS. Neurology. 2022;99(7):e743‐e750.
Tilanus TBM, Groothuis JT, Ten Broek‐Pastoor JMC, et al. Respiratory assessment of ALS patients: a Nationwide survey of current Dutch practice. J Neuromuscul Dis. 2018;5:431‐438. doi:10.3233/JND-18302
Lechtzin 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.
Sferrazza Papa GF, Pellegrino GM, Shaikh H, Lax A, Lorini L, Corbo M. Respiratory muscle testing in amyotrophic lateral sclerosis: a practical approach. Minerva Med. 2018;109(6 Suppl 1):11‐19.
Pirola A, De Mattia E, Lizio A, et al. The prognostic value of spirometric tests in amyotrophic lateral sclerosis patients. Clin Neurol Neurosurg. 2019;184:105456.
EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis, Andersen PM, Abrahams S, et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)‐‐revised report of an EFNS task force. Eur J Neurol. 2012;19(3):360‐375.
Calvo A, Vasta R, Moglia C, et al. Prognostic role of slow vital capacity in amyotrophic lateral sclerosis. J Neurol. 2020;267(6):1615‐1621.
Manera U, Torrieri MC, Moglia C, et al. The role of arterial blood gas analysis (ABG) in amyotrophic lateral sclerosis respiratory monitoring. J Neurol Neurosurg Psychiatry. 2020;91(9):999‐1000.
Boentert M, Glatz C, Helmle C, Okegwo A, Young P. Prevalence of sleep apnoea and capnographic detection of nocturnal hypoventilation in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 2018;89(4):418‐424.
Reyhani A, Benbir Senel G, Karadeniz D. Effects of sleep‐related disorders on the prognosis of amyotrophic lateral sclerosis. Neurodegener Dis. 2019;19(3–4):148‐154.
Brooks BR, Miller RG, Swash M, Munsat TL, World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000;1:293‐299. doi:10.1080/146608200300079536
Schito P, Ceccardi G, Calvo A, et al. Clinical features and outcomes of the flail arm and flail leg and pure lower motor neuron MND variants: a multicentre Italian study. J Neurol Neurosurg Psychiatry. 2020;91(9):1001‐1003.
Chiò A, Calvo A, Moglia C, Mazzini L, Mora G, Group* P study. Phenotypic heterogeneity of amyotrophic lateral sclerosis: a population based study. J Neurol Neurosurg Psychiatry. 2011;82(7):740‐746.
Cedarbaum JM, Stambler N, Malta E, et al. The ALSFRS‐R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS study group (phase III). J Neurol Sci. 1999;169(1–2):13‐21.
Trojsi F, Siciliano M, Femiano C, et al. Comorbidity of dementia with amyotrophic lateral sclerosis (ALS): insights from a large multicenter Italian cohort. J Neurol. 2017;264:2224‐2231. doi:10.1007/s00415-017-8619-4
Labra J, Menon P, Byth K, Morrison S, Vucic S. Rate of disease progression: a prognostic biomarker in ALS. J Neurol Neurosurg Psychiatry. 2016;87(6):628‐632.
Manera U, Torrieri MC, Moglia C, et al. Arterial blood gas analysis: base excess and carbonate are predictive of noninvasive ventilation adaptation and survival in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. 2021;22(sup1):33‐39. doi:10.1080/21678421.2021.1887263
Orlikowski D, Prigent H, Quera Salva M‐A, et al. Prognostic value of nocturnal hypoventilation in neuromuscular patients. Neuromuscul Disord. 2017;27(4):326‐330.
Roussos C, Macklem PT. The respiratory muscles. N Engl J Med. 1982;307(13):786‐797.
Annane D, Quera‐Salva MA, Lofaso F, et al. Mechanisms underlying effects of nocturnal ventilation on daytime blood gases in neuromuscular diseases. Eur Respir J. 1999;13(1):157‐162.
Elman LB, Siderowf AD, McCluskey LF. Nocturnal oximetry: utility in the respiratory Management of Amyotrophic Lateral Sclerosis. Am J Phys Med Rehabil. 2003;82(11):866‐870.
Quaranta VN, Carratù P, Damiani MF, et al. The prognostic role of obstructive sleep apnea at the onset of amyotrophic lateral sclerosis. Neurodegener Dis. 2017;17(1):14‐21.
Czaplinski 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.
Alarcan H, Cotet C, Lépine N, et al. Evaluation of arterial blood gas parameters as prognostic markers in amyotrophic lateral sclerosis. Eur J Neurol. 2023;30(6):1611‐1618.
Velasco R, Salachas F, Munerati E, et al. Nocturnal oxymetry in patients with amyotrophic lateral sclerosis: role in predicting survival. Rev Neurol (Paris). 2002;158(5 Pt 1):575‐578.
Morelot‐Panzini C, Bruneteau G, Gonzalez‐Bermejo J. NIV in amyotrophic lateral sclerosis: the “when” and “how” of the matter. Respirology. 2019;24(6):521‐530.
Schiffman PL, Belsh JM. Pulmonary function at diagnosis of amyotrophic lateral sclerosis: rate of deterioration. Chest. 1993;103(2):508‐513.