Noninvasive mechanical ventilation assistance in amyotrophic lateral sclerosis: a systematic review

Authors

Keywords:

Amyotrophic lateral sclerosis, Respiratory insufficiency, Noninvasive ventilation, Survival, Quality of life, Motor neuron diseases, Respiratory failure, Hypoventilations, Artificial respiration, Health system, Health, attitude to

Abstract

BACKGROUND: Respiratory failure is the most common cause of death in patients with amyotrophic lateral sclerosis (ALS), and morbidity is related to poor quality of life (QOL). Non-invasive ventilation (NIV) may be associated with prolonged survival and QOL in patients with ALS.
OBJECTIVES: To assess whether NIV is effective and safe for patients with ALS in terms of survival and QOL, alerting the health system.
DESIGN AND SETTING: Systematic review was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses reporting standards using population, intervention, comparison, and outcome strategies.
METHODS: The Cochrane Library, CENTRAL, MEDLINE, LILACS, EMBASE, and CRD databases were searched based on the eligibility criteria for all types of studies on NIV use in patients with ALS published up to January 2022. Data were extracted from the included studies, and the findings were presented using a narrative synthesis.
RESULTS: Of the 120 papers identified, only 14 were related to systematic reviews. After thorough reading, only one meta-analysis was considered eligible. In the second stage, 248 studies were included; however, only one systematic review was included. The results demonstrated that NIV provided relief from the symptoms of chronic hypoventilation, increased survival, and improved QOL compared to standard care. These results varied according to clinical phenotype.
CONCLUSIONS: NIV in patients with ALS improves the outcome and can delay the indication for tracheostomy, reducing expenditure on hospitalization and occupancy of intensive care unit beds.
SYSTEMATIC REVIEW REGISTRATION: PROSPERO database: CRD42021279910 — https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=279910.

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Author Biographies

Carolina da Cunha-Correia, Universidade de Pernambuco, Recife, Pernambuco, Brazil

PhD. Neurologist and Professor, Department of Neurology, Hospital Universitário Oswaldo Cruz (HUOC), Universidade de Pernambuco (UPE), Recife (PE), Brazil.

Mylana Dandara Pereira Gama, Universidade de Pernambuco, Recife, Pernambuco, Brazil

MD. Resident Physician, Department of Neurology, Hospital Universitário Oswaldo Cruz (HUOC), Universidade de Pernambuco (UPE), Recife (PE), Brazil.

Pedro Nogueira Fontana, Universidade de Pernambuco, Recife, Pernambuco, Brazil

MSc. Neurologist, Department of Neurology, Hospital Universitário Oswaldo Cruz (HUOC), Universidade de Pernambuco (UPE), Recife (PE), Brazil.

Francisca Goreth Malheiro Moraes Fantini, Santa Casa de Misericórdia de Fernandópolis, Fernandópolis, São Paulo, Brazil.

MD. Neurologist and Neurophysiologist, Santa Casa de Misericórdia de Fernandópolis (SCF), Fernandópolis (SP), Brazil.

Gilmar Fernandes Prado, Universidade Federal de São Paulo, São Paulo, São Paulo, Brazil

PhD. Neurologist and Professor, Department of Medicine, Escola Paulista de Medicina (EPM), Universidade Federal de São Paulo (UNIFESP), São Paulo (SP), Brazil.

Mário Emílio Teixeira Dourado Júnior, Universidade Federal do Rio Grande do Norte, Natal, Rio Grande do Norte, Brazil

PhD. Neurologist and Professor, Department of Integrated Medicine, Centro de Ciências da Saúde (CCS), Universidade Federal do Rio Grande do Norte (UFRN), Natal (RN), Brazil.

Paulo Adriano Schwingel, Universidade de Pernambuco, Recife, Pernambuco, Brazil

PhD. Sports Physiologist and Associate Professor, Human Performance Research Laboratory, Universidade de Pernambuco (UPE), Petrolina (PE), Brazil.

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Published

2025-03-24

How to Cite

1.
Cunha-Correia C da, Gama MDP, Fontana PN, Fantini FGMM, Prado GF, Dourado Júnior MET, Schwingel PA. Noninvasive mechanical ventilation assistance in amyotrophic lateral sclerosis: a systematic review. Sao Paulo Med J [Internet]. 2025 Mar. 24 [cited 2025 May 9];142(1):1-10. Available from: https://periodicosapm.emnuvens.com.br/spmj/article/view/2984

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