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Is survival improved by the use of NIV and PEG in amyotrophic lateral sclerosis (ALS)?: A post-mortem study of 80 ALS patients
Umeå University, Faculty of Medicine, Department of Pharmacology and Clinical Neuroscience.
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2017 (English)In: PLoS ONE, ISSN 1932-6203, E-ISSN 1932-6203, Vol. 12, no 5, e0177555Article in journal (Refereed) Published
Abstract [en]

Background: Non-invasive ventilation (NIV) and percutaneous gastrostomy (PEG) are guideline-recommended interventions for symptom management in amyotrophic lateral sclerosis (ALS). Their effect on survival is controversial and the impact on causes of death is unknown.

Objective: To investigate the effect of NIV and PEG on survival and causes of death in ALS patients.

Methods: Eighty deceased ALS patients underwent a complete post mortem analysis for causes of death between 2003 and 2015. Forty-two of these patients consented for genetic testing. Effects of NIV and PEG on survival and causes of death were analyzed in a multivariable Cox proportional hazard regression.

Results: Six patients, who requested assisted suicide causing drug-induced hypoxia, were excluded from final analysis. Respiratory failure was the main cause of death in 72 out of 74 patients. Fifteen out of 74 died of aspiration pneumonia 23/74 of bronchopneumonia and 8/74 of a combination of aspiration pneumonia and bronchopneumonia. Twenty died of hypoxia without concomitant infection, and six patients had pulmonary embolism alone or in combination with pneumonia. NIV (p = 0.01) and PEG (p<0.01) had a significant impact on survival. In patients using NIV bronchopneumonia was significantly more frequent (p <0.04) compared to non-NIV patients. This effect was even more pronounced in limb onset patients (p<0.002). Patients with C9orf72 hexanucleotide repeat expansions showed faster disease progression and shorter survival (p = 0.01).

Conclusion: The use of NIV and PEG prolongs survival in ALS. This study supports current AAN and EFNS guidelines which recommend NIV and PEG as a treatment option in ALS. The risk of bronchopneumonia as cause of death may be increased by NIV.

Place, publisher, year, edition, pages
2017. Vol. 12, no 5, e0177555
National Category
Neurology
Identifiers
URN: urn:nbn:se:umu:diva-136332DOI: 10.1371/journal.pone.0177555ISI: 000402058800019OAI: oai:DiVA.org:umu-136332DiVA: diva2:1113142
Available from: 2017-06-21 Created: 2017-06-21 Last updated: 2017-06-21Bibliographically approved

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Andersen, Peter M.
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CiteExportLink to record
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Citation style
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