- •Respiratory tract infections can lead to an acute respiratory failure in ALS.
- •Acute episodes in ALS without severe bulbar dysfunction can be managed with NIV.
- •Norris bulbar score can predict NIV failure in ALS during acute chest episodes.
Amyotrophic lateral sclerosis (ALS) patients can suffer episodes of lower respiratory tract infections (LRTI) leading to an acute respiratory failure (ARF) requiring noninvasive ventilation (NIV).
To determine whether clinical or functional parameters can predict noninvasive management failure during LRTI causing ARF in ALS.
Material and method
A prospective study involving all ALS patients with ARF requiring NIV in a Respiratory Care Unit. NIV was provided with volume-cycled ventilators.
63 ALS patients were included (APACHE II: 14.93 ± 3.56, Norris bulbar subscore (NBS): 18.78 ± 9.68, ALSFRS-R: 19.90 ± 6.98, %FVC: 40.01 ± 18.07%, MIC: 1.62 ± 0.74 L, PCF 2.51 ± 1.15 L/s, PImax –34.90 ± 19.44 cm H2O, PEmax 51.20 ± 28.84 cm H2O). In 73.0% of patients NIV was successful in averting death or endotracheal intubation. Differences were found between the success and failure in the NBS (22.08 ± 6.15 vs 8.66 ± 3.39, p < 0.001), ALSFRS (22.08 ± 6.11 vs 12.71 ± 4.39, p < 0.001), PCFMI-E (3.85 ± 0.77 vs 2.81 ± 0.91 L/s, p = 0.007) and ALS onset (spinal/bulbar 33/13 vs 7/10, p = 0.03). The predictor of NIV failure was the NBS (OR 0.53, 95% CI 0.31–0.92, p 0.002) with a cut-off point of 12 (S 0.93; E 0.97; PPV 0.76; NPV 0.97).
NBS can predict noninvasive management failure during LRTI in ALS.
Abbreviations:AB (acute bronchitis), ALS (amyotrophic lateral sclerosis), ALSFRS-R (revised amyotrophic lateral sclerosis functional rating scale), APACHE II (Acute Physiology and Chronic Health Evaluation), ARF (acute respiratory failure), AUC (area under the curve), CAP (community acquired pneumonia), COPD (chronic obstructive pulmonary disease), BMI (body mass index), ETI (endotracheal intubation), FVC (forced vital capacity), %FVC (predicted FVC), ICU (intensive care unit), LRTI (lower respiratory tract infection), MAC (mechanically assisted cough), MIC (maximum insufflation capacity), NBS (Norris bulbar subscore), NIV (noninvasive ventilation), NMD (neuromuscular disease), PCF (peak cough flow), PCFMIC (manually assisted PCF), PCFMI-E (mechanically assisted PCF), PEmax (maximum expiratory pressure), PImax (maximum inspiratory pressure), RCU (respiratory care unit), ROC (receiver operating characteristic), RR (respiratory rate), SBI (severe bulbar impairment), TMV (tracheotomy mechanical ventilation)
To read this article in full you will need to make a payment
Purchase one-time access:Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
One-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:Subscribe to Journal of the Neurological Sciences
Already a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
- Hospitalization in amyotrophic lateral sclerosis: causes, costs and outcomes.Neurology. 2001; 56: 753-757
- Oximetry and indications for tracheotomy for amyotrophic lateral sclerosis.Chest. 2004; 126: 1502-1507
- Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial.Lancet Neurol. 2006; 5: 140-147
- Chronic respiratory care for neuromuscular diseases in adults.Eur. Respir. J. 2009; 34: 444-451
- Appropiate management of respiratory problems is of utmost importance in the treatment of patients with amyotrophic lateral sclerosis.Chest. 2005; 127: 1879-1882
- Acute applications of noninvasive positive pressure ventilation.Chest. 2003; 124: 699-713
- BTS Guideline. Non-invasive ventilation in acute respiratory failure.Thorax. 2002; 57: 192-211
- Amyotrophic lateral sclerosis: successful treatment of mucus plugging by mechanical insufflation-exsufflation.Am. J. Phys. Med. Rehabil. 1997; 76: 338-339
- Alternatives to endotracheal intubation for patients with neuromuscular diseases.Am. J. Phys. Med. Rehabil. 2005; 84: 851-857
- Mechanical insufflation-exsufflation improves outcomes for neuromuscular disease patients with respiratory tract infections.Am. J. Phys. Med. Rehabil. 2005; 84: 83-88
- Noninvasive ventilatory approach to treatment of acute respiratory failure in neuromuscular disorders. A comparison with endotracheal intubation.Intensive Care Med. 2000; : 26384-26390
- Home tracheotomy mechanical ventilation in patients with amyotrophic lateral sclerosis: causes, complications and 1-year survival.Thorax. 2011; 66: 948-952
- 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
- Guidelines for the management of adult lower respiratory tract infections.Clin. Microbiol. Infect. 2011; 17: E1-E59
- Predictors of ineffective cough during a chest infection in patients with stable amyotrophic lateral sclerosis.Am. J. Respir. Crit. Care Med. 2007; 175: 1266-1271
- The ALSFRS-R: a revised ALS function rating scale that incorporates assessment of respiratory function. DNF ALS study group (Phase III).J. Neurol. Sci. 1999; 169: 13-21
- A double-blind, placebo-controlled trial of high doses of gangliosides in amyotrophic lateral sclerosis.Neurology. 1989; 39: 1635-1637
- APACHE II: a severity of disease classification system.Crit. Care Med. 1985; 13: 818-829
- Efficacy of mechanical insufflation-exsufflation in extubating unweanable subjects with restrictive pulmonary disorders.Respir. Care. 2015; 60: 477-483
- Causes and outcomes of acute neuromuscular respiratory failure.Arch. Neurol. 2010; 67: 1089-94
- Effect of upper respiratory tract infection in patients with neuromuscular disease.Am. J. Respir. Crit. Care Med. 1997; 156: 659-664
- Noninvasive management of an acute chest infection for a patient with ALS.J. Neurol. Sci. 2003; 209: 111-113
- BiPAP in acute respiratory failure due to myasthenic crisis may prevent intubation.Neurology. 2002; 59: 1647-1649
- Efficacy of mechanical insufflation–exsufflation in extubating unweanable subjects with restrictive pulmonary disorders.Respir. Care. 2015; 60: 621-622
- Non-invasive ventilation in community-acquired pneumonia and severe acute respiratory failure.Intensive Care Med. 2012; 38: 458-466
- Non-invasive positive pressure ventilation to treat respiratory failure resulting from exacerbations of chronic obstructive pulmonary disease: Cochrane systematic review and meta-analysis.BMJ. 2003; 326: 185
- Noninvasive mechanical ventilation in acute respiratory failure due to chronic obstructive pulmonary disease: correlates for success.Thorax. 1995; 50: 755-757
- Nasal mechanical ventilation for hypercapnic respiratory failure in chronic obstructive pulmonary disease: determinants of success and failure.Crit. Care Med. 1994; 22: 1253-1261
- Predicting the result of noninvasive ventilation in severe acute exacerbations of patients with chronic airflow limitation.Chest. 2000; 117: 828-832
- Spinal muscular atrophy type 1: prolongation of survival by noninvasive respiratory aids.Pediatr. Asthma Allergy Immunol. 2009; 22: 151-162
- Noninvasive ventilation effectiveness and the effect of ventilatory mode on survival in ALS patients.Amyotroph. Lateral Scler. Frontotemporal Degener. 2014; 15: 55-61
- Oropharyngeal dysphagia in amyotrophic lateral sclerosis: neurological and dysphagia specific rating scales.Amyotroph. Lateral Scler. Other Motor Neuron Disord. 2004; 5: 150-153
- Noninvasive ventilation and the upper airway: should we pay more attention?.Crit. Care. 2015; 11: 139-147
- Noninvasive positive pressure ventilation in acute respiratory failure.Am. J. Respir. Crit. Care Med. 2001; 163: 283-291
Published online: August 21, 2015
Accepted: August 18, 2015
Received in revised form: August 14, 2015
Received: April 17, 2015
☆Authors' contributions: ES, JS and PB conception and design of the study, acquisition of data, analysis and interpretation of data, drafting the article and final approval of the submitted version. JM — drafting the article and final approval of the submitted version.
© 2015 Elsevier B.V. Published by Elsevier Inc. All rights reserved.