Do patients with amyotrophic lateral sclerosis (ALS) have increased energy needs?

Vaisman, Nachum; Lusaus, Michal; Nefussy, Beatrice; Niv, Eva; Comaneshter, Doron; Hallack, Ron; Drory, Vivian E.

doi:10.1016/j.jns.2008.12.027

Cited by 108 publications

(64 citation statements)

References 20 publications

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“…5,6 Clinical observations consistently report that patients with ALS are generally lean with a normal or low body mass index (BMI). [7][8][9] They typically lose muscle mass, bone density, and body fat, and consequently weight, as the disease progresses, 8,[10][11][12] and although neurogenic muscle wasting is the main pathophysiologic mechanism explaining this, their energy stores are also decreased. 13 Several lines of evidence have indicated that patients with ALS have an increased metabolism with higher energy expenditure than energy intake.…”

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confidence: 99%

Prediagnostic body fat and risk of death from amyotrophic lateral sclerosis

et al. 2013

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Objectives: The aim of this study was to investigate for the first time the association between body fat and risk of amyotrophic lateral sclerosis (ALS) with an appropriate prospective study design. Methods:The EPIC (European Prospective Investigation into Cancer and Nutrition) study included 518,108 individuals recruited from the general population across 10 Western European countries. At recruitment, information on lifestyle was collected and anthropometric characteristics were measured. Cox hazard models were fitted to investigate the associations between anthropometric measures and ALS mortality.Results: Two hundred twenty-two ALS deaths (79 men and 143 women) occurred during the followup period (mean follow-up 5 13 years). There was a statistically significant interaction between categories of body mass index and sex regarding ALS risk (p 5 0.009): in men, a significant linear decrease of risk per unit of body mass index was observed (hazard ratio 5 0.93, 95% confidence interval 0.86-0.99 per kg/m 2 ); among women, the risk was more than 3-fold increased for underweight compared with normal-weight women. Among women, a significant risk reduction increasing the waist/hip ratio was also evident: women in the top quartile had less than half the risk of ALS compared with those in the bottom quartile (hazard ratio 5 0.48, 95% confidence interval 0.25-0.93) with a borderline significant p value for trend across quartiles (p 5 0.056). Conclusion:Increased prediagnostic body fat is associated with a decreased risk of ALS mortality.

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Prediagnostic body fat and risk of death from amyotrophic lateral sclerosis

et al. 2013

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“…Amyotrophic Lateral Sclerosis (ALS) is another condition where PEG is routinely employed (James et al, 1998;James et al, 2005;Rimon et al, 2005;Mitsumoto et al, 2003). In addition to progressive issues with dysphagia, ALS patients have increased energy needs, and it has been suggested that PEG can play an important role in preventing additional muscle loss (Desport et al, 1999;Spataro et al, 2011;Vaisman et al, 2009). Recent guidelines recommend ALS patients receive PEG when oral intake is limited and body weight begins to decline (Andersen et al, 2007;Radunovic et al, 2007;Spataro et al, 2011).…”

Section: Indications For Percutaneous Endoscopic Gastrostomymentioning

confidence: 99%

Percutaneous Endoscopic Gastrostomy in Neurological Patients

Burke¹,

Geller²

2011

Gastrostomy

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“…One such condition is amyotrophic lateral sclerosis (ALS), which shares a clinical and pathological overlap with FTD and where consistent changes have been found (Ahmed et al, 2016b), including increased resting energy expenditure in up to 50% of patients (Bouteloup et al, 2009;Vaisman et al, 2009). It has also been suggested that the hypermetabolic state (defined as increased energy expenditure) is intrinsically linked to the process of neurodegeneration with several animal models of TAR DNA-binding protein 43 (TDP-43, encoded by TARDBP) and the C9orf72 gene expansion exhibiting hypermetabolism and weight loss (Dupuis et al, 2004;Chiang et al, 2010;Shan et al, 2010;Xu et al, 2010;Koppers et al, 2015).…”

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confidence: 99%

Energy expenditure in frontotemporal dementia: a behavioural and imaging study

Landín-Romero

Collette

Klaauw

et al. 2017

J Neurol Neurosurg Psychiatry

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See Finger (doi:10.1093/aww312) for a scientific commentary on this article.Abnormal eating behaviour and metabolic parameters including insulin resistance, dyslipidaemia and body mass index are increasingly recognized as important components of neurodegenerative disease and may contribute to survival. It has previously been established that behavioural variant frontotemporal dementia is associated with abnormal eating behaviour characterized by increased sweet preference. In this study, it was hypothesized that behavioural variant frontotemporal dementia might also be associated with altered energy expenditure. A cohort of 19 patients with behavioural variant frontotemporal dementia, 13 with Alzheimer's disease and 16 (age-and sex-matched) healthy control subjects were studied using Actiheart devices (CamNtech) to assess resting and stressed heart rate. Actiheart devices were fitted for 7 days to measure sleeping heart rate, activity levels, and resting, active and total energy expenditure. Using high resolution structural magnetic resonance imaging the neural correlates of increased resting heart rate were investigated including cortical thickness and region of interest analyses. In behavioural variant frontotemporal dementia, resting (P = 0.001), stressed (P = 0.037) and sleeping heart rate (P = 0.038) were increased compared to control subjects, and resting heart rate (P = 0.020) compared to Alzheimer disease patients. Behavioural variant frontotemporal dementia was associated with decreased activity levels compared to controls (P = 0.002) and increased resting energy expenditure (P = 0.045) and total energy expenditure (P = 0.035). Increased resting heart rate correlated with behavioural (Cambridge Behavioural Inventory) and cognitive measures (Addenbrooke's Cognitive Examination). Increased resting heart rate in behavioural variant frontotemporal dementia correlated with atrophy involving the mesial temporal cortex, insula, and amygdala, regions previously suggested to be involved exclusively in social and emotion processing in frontotemporal dementia. These neural correlates overlap the network involved in eating behaviour in frontotemporal dementia, suggesting a complex interaction between eating behaviour, autonomic function and energy homeostasis. As such the present study suggests that increased heart rate and autonomic changes are prevalent in behavioural variant frontotemporal dementia, and are associated with changes in energy expenditure. An understanding of these changes and neural correlates may have potential relevance to disease progression and prognosis. Correspondence may also be addressed to: Professor John Hodges, e-mail: j.hodges@neura.edu.au Keywords: frontotemporal dementia; metabolism; heart rate; physiology; autonomic function Abbreviations: ALS = amyotrophic lateral sclerosis; BMI = body mass index; bvFTD = behavioural variant frontotemporal dementia; FTD = frontotemporal dementia; RMSSD = root mean squared of successive differences

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Do patients with amyotrophic lateral sclerosis (ALS) have increased energy needs?

Cited by 108 publications

References 20 publications

Prediagnostic body fat and risk of death from amyotrophic lateral sclerosis

Prediagnostic body fat and risk of death from amyotrophic lateral sclerosis

Percutaneous Endoscopic Gastrostomy in Neurological Patients

Energy expenditure in frontotemporal dementia: a behavioural and imaging study

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