Factors related to respiration influencing survival and respiratory function in patients with amyotrophic lateral sclerosis: a retrospective study

Leonardis, Lea; Grošelj, Leja Dolenc; Vidmar, Gaj

doi:10.1111/j.1468-1331.2012.03754.x

Cited by 25 publications

(19 citation statements)

References 26 publications

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“…The observed benefits of NIV on pulmonary function decline corroborate previous literature 20 22. While the benefit was significant overall for FVC, FEV 1 , MIP and MEP, we found a wide variability in magnitude of effects across pulmonary function tests and phenotypes.…”

Section: Discussionsupporting

confidence: 90%

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Identifying who will benefit from non-invasive ventilation in amyotrophic lateral sclerosis/motor neurone disease in a clinical cohort

Berlowitz

Howard

Fiore

et al. 2015

J Neurol Neurosurg Psychiatry

113

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Between 1991 and 2011, 1198 patients diagnosed with ALS/MND were registered. 929 patients (77.5%) fulfilled the selection criteria and their data were analysed. Median tracheostomy free survival from symptom onset was 28 months in NIV-treated patients compared to 15 months in untreated (Univariate Cox regression HR=0.61 (0.51 to 0.73), p<0.001). The positive survival effect of NIV persisted when the model was adjusted for age, gender, riluzole and percutaneous endoscopic gastrostomy use (HR=0.72 (0.60 to 0.88, p=0.001). In contrast with the only randomised controlled trial, NIV statistically significantly increased survival by 19 months in those with ALS-bulbar onset (Univariate HR=0.50 (0.36 to 0.70), multivariate HR=0.59 (0.41 to 0.83)). These data confirm that NIV improves survival in MND/ALS. The overall magnitude of benefit is 13 months and was largest in those with ALS-bulbar disease. Future research should explore the optimal timing of NIV initiation within phenotypes in order to optimise respiratory function, quality of life and survival.

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Section: Discussionsupporting

confidence: 90%

“…Importantly, when not accounting for left truncation, observational studies on NIV in MND maybe be biased towards favourable survival in patients who did not use NIV. Therefore, the real survival benefits of NIV may be larger than the reported in previous cohort studies 3 20 22…”

Section: Discussionmentioning

confidence: 90%

Identifying who will benefit from non-invasive ventilation in amyotrophic lateral sclerosis/motor neurone disease in a clinical cohort

Berlowitz

Howard

Fiore

et al. 2015

J Neurol Neurosurg Psychiatry

113

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“…Of note, the accelerated muscle wasting due to hypermetabolism is bound to involve respiratory muscles. A NIV-related reduction in energy expenditure could therefore theoretically contribute to slow respiratory decline, yet a recent retrospective study suggested, apparently for the first time, that NIV could indeed decrease the ALS-related decline of VC [42]. The correlation observed between REE and FVC and Pi MAX in our patients (Table 3) tends to supports this hypothesis.…”

Section: Discussionsupporting

confidence: 65%

Noninvasive ventilation reduces energy expenditure in amyotrophic lateral sclerosis

et al. 2014

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BackgroundAmyotrophic lateral sclerosis (ALS) leads to chronic respiratory failure. Diaphragmatic dysfunction, a major driver of dyspnea and mortality, is associated with a shift of the burden of ventilation to extradiaphragmatic inspiratory muscles, including neck muscles. Besides, energy expenditure is often abnormally high in ALS, and this is associated with a negative prognostic value. We hypothesized that noninvasive ventilation (NIV) would relieve inspiratory neck muscles and reduce resting energy expenditure (REE).MethodsUsing indirect calorimetry, we measured REE during spontaneous breathing (REESB) and NIV (REENIV) in 16 ALS patients with diaphragmatic dysfunction, during the first 3 months of NIV. Measured values were compared with predicted REE (REEpred)(Harris-Benedict equation).ResultsNIV abolished inspiratory neck muscle activity. Even though our patients were not hypermetabolic, on the contrary, with a REESB that was lower than REEpred (average 11%), NIV did reduce energy expenditure. Indeed, median REENIV, in this population with a mean body mass index of 21.4 kg.m-2, was 1149 kcal/24 h [interquartile 970-1309], lower than REESB (1197 kcal/24 h, 1054-1402; mean difference 7%; p = 0.03, Wilcoxon). REESB and REENIV were correlated with forced vital capacity and maximal inspiratory pressure.ConclusionsNIV can reduce energy expenditure in ALS patients probably by alleviating the ventilatory burden imposed on inspiratory neck muscles to compensate diaphragm weakness. It remains to be elucidated whether or not, in which population, and to what extent, NIV can be beneficial in ALS through the corresponding reduction in energy expenditure.

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“…In our retrospective study [1], 60 out of 189 patients with amyotrophic lateral sclerosis (ALS) had presented the predominantly bulbar form at disease onset. The disease form was defined according to the patient's history of first symptom and, if there was uncertain history, the patients were assigned in groups according to the predominant clinical signs at first examination (bulbar or pseudobulbar signs versus lower or upper motor neuron deficit on limbs).…”

Section: Sirsmentioning

confidence: 99%

Non‐invasive positive‐pressure ventilation in patients with amyotrophic lateral sclerosis: spinal versus bulbar form

Leonardis

Grošelj

2013

Euro J of Neurology

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Factors related to respiration influencing survival and respiratory function in patients with amyotrophic lateral sclerosis: a retrospective study

Abstract: Any respiratory abnormality should prompt the clinician to start discussing NIV with the patient. NIV prolongs survival and improves respiratory function.

Cited by 25 publications

References 26 publications

Identifying who will benefit from non-invasive ventilation in amyotrophic lateral sclerosis/motor neurone disease in a clinical cohort

Identifying who will benefit from non-invasive ventilation in amyotrophic lateral sclerosis/motor neurone disease in a clinical cohort

Noninvasive ventilation reduces energy expenditure in amyotrophic lateral sclerosis

Non‐invasive positive‐pressure ventilation in patients with amyotrophic lateral sclerosis: spinal versus bulbar form

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